A Breakdown of Trust in Our Patient/Physician Relationship.

 

Last night, after we changed banzel from liquid to tablet and increased depakene, per neuro orders, I think he absorbs the liquid form better.  We haven't seen these seizures in a while. 

Is it strange that unlike any of my other blog posts, I feel anxious, nervous and almost paranoid that someone is going to come get me and tear a rip into the nice tapestry of my little cozy life after reading this? But, I'll be brave and write it anyway. 

Yesterday, I had a follow up video appointment with Lucas' new neurologist who was assigned to us after his other neurologist retired.  This was only our second appointment. We discussed Lucas seizure control, or the lack thereof, and change in medication dosage.  We agreed about a month ago that we would increase his medication from 2 tablets twice a day to 4.  He asked if Lucas still experienced seizures and yes, he still does once or twice a day usually triggered by waking up in the morning. We slowly titrated it up to 4 but wasn't seeing much improvement and in fact he had a major fall at school from a seizure which landed him in the ER.  (See previous entry) I worried the new dosage may actually be exacerbating rather than reducing his seizures so we titrated it back down to 2 tablets. What is interesting is we never requested an increase in meds and considered ourselves very fortunate that he only has 2-3 seizures in comparison to other children with this syndrome who experience multiple clusters and more severe forms of seizures per day. Daily seizures for a typical child is definitely troubling but for a rare child like Lucas, it sometimes is a given. The ER lab work showed that he was in the therapeutic range for the drug. I prepared to relay to him what had happened at school during our meeting yesterday, but at one point the topic of reporting me to child protective services came up and my mind just went blank, my trust for him went out the door and I was hesitant to share any further information with him. When I honestly told him why we did not increase the med as he prescribed, he seemed very irritated and even mentioned that child protective services can be called if I am not giving him the prescribed dose from a physician.   

We have been dealing with seizures for so long that having one or two seizures doesn't warrant an immediate consultation. His seizures come and go, he's had all types and they morph into new ones which fade away and sometimes I honestly believe they are independent of the medications we give and do very little to affect how, when and the frequency of their appearance, except for banzel  (rufinammide). The seizure triggers are a mystery except for the ones he gets just after waking up in the morning and when he has gone a long stretch of time without them, he is prone to get clusters.  He failed 3 other meds before which is typical of kids with this syndrome.  One person had been on multiple AED’s, had more than one VNS implanted, had  corpus callosotomy, sought out tests from Mayo clinic and in his mid 30’s show constant seizure activities detected on an EEG. We feel fortunate that Luki has some quality of life, not zoned out on too many meds or on G tube feeds, npo status for life, or dealing with bowel impaction or psych issues, etc.. that can manifest with this syndrome.  But who knows what can happen in the future? Some parents report that their children experience paradoxical effects to meds or get the rare side effect that most people do not experience.  If the chances are one in a million, they say their child will get it.  When an increased medication dose correlates to injury, it scares me from maintaining that dose. Yes, I could have sent the neurologist an email him sooner about why we decided to bring the dose down, but I didn't and that may be on me.  Lesson learned.  However, it was so upsetting for him to bring up CPS as a scare tactic to get compliance.  My whole world revolves around my boys and I may not be perfect but bringing out the threat of possibly having custody of my children taken away is a bit draconian.  Wouldn't any thinking parent who sees that increase in a med has landed their child in the ER with a bloody gash in their face for the first time ever in their life consider doing the same?  These are powerful drugs and need to be titrated slowly and cautiously and if an increase worsens seizures, titrating back down seems safer thing to do at the time.  Couldn't he just have said, "next time this happens let me know what you do. and we can move forward together as a team." or something to that effect? I do what I do for my child out of love, not out of fear of the CP and leveraging the latter to get me to give the best care is so demeaning. 

I, too am a mandated reporter first as a volunteer at my son’s school and also as a registered nurse. ( Certificate of Completion from Mandated Reporter Training). I have taken a two-hour training class on what situations are appropriate to report which predominantly involves various forms of abuse, neglect and child trafficking.  Using that power to affect a family negatively without judicious thought and consideration for the family is an abuse of power. - this point was an actual module of the course.  A mother of a child with Dup15q shared that a gastroenterologist once recommended a surgery for her child for which she was in disagreement. The doctor threatened reporting her to CPS and out of fear, scheduled a consultation with a surgeon who agreed with the mother that the surgery was unnecessary.  Afterwards, she fired her GI doctor.  Threats like this undermines medical freedom for parents and a trust-based relationship between patient and physician. 

Does the physician know best in all situations and their judgement supersedes that of the parent?  Always?  A head and neck surgeon once said my other son needed surgery in his ear when he was a toddler, I disagreed. I never followed up and he is perfectly fine now. Outside of the Dup15q clinic physicians, researchers and parents who are familiar with the condition, most have not even heard of the syndrome. Everyone has a right to refuse treatment but in the case of a minor, if there is a difference of opinion between the physician and the parent, does the former's legally carry more weight? I’ve lived with my son for 15 years every day and know that when his poop comes out a certain way he is lacking fluids. This neurologist has never even seen my son and this was only our second video appointment.  Parents aren't all clueless about what is medically happening with their children. Of course, there are exceptions which is why doctors need to be cautiously sensitive and assess each situation individually instead of targeting all parents who refuse their medically trained recommendation.  Anything other than that is a bruised ego trying to reclaim itself. 

I'm sure the neurologist feels he is doing his part well and as a mandated reporter for child abuse, he has the authority to do so. And I am sure I frustrate him along with the uncertainty of dealing with a rare condition.  In situations like this, it is best to not piss these doctors off by provoking them.   They do get retaliatory and it reminds me of the mom who had her child with a rare condition taken hostage by the hospital and ultimately committed suicide due to years of frustration and injustice.  I've never sympathized more with parents of children with rare conditions and intellectual disability as these kids often have no sense of danger, tend to elope, injure themselves from seizures, self-harm and if they encounter an unempathetic doctor who dislike the parents for differing philosophy in medical care, they can easily be reported to the state.  The doctor also said if my son injured himself from a fall and he landed in the ER but was not taking the exact prescribed dose, the physician can contact CPS against the parent for negligence. If he is injured even on the prescribed dose of medication can a parent report the physician to CPS? I think most rational doctors would not go there unless there were other signs of abuse/neglect, but some would and who needs the added stress and inconvenience of having to deal the CPS on top of managing the care of a medically complex child who requires total care in all activities of daily living?

What this experience highlights for me is the importance of trust in a physician/patient relationship.  I have requested our primary pediatrician for a referral to another neurologist because regardless of how knowledgeable a physician is, and how gung-ho they are to manage my son's seizures, when trust is broken, the relationship becomes dangerous, especially that in which power is not equal.  

I can see why there were so many COVID naysayers and antivaxxers because somewhere along the line, patients' voices were not respected, their unique needs were minimized so much so that when the anger and frustration reached critical mass, a whole movement and confusion was unleashed into all of society.  And I attribute all that, at its core, to the breakdown in patient/physician trust.  

Palliative ASM.

 A palliative approach to seizure control means treating seizures primarily to maximize comfort, dignity, and quality of life, rather than pursuing complete seizure elimination at all costs.

It is most often used when someone has a serious, progressive, or terminal illness, or when seizures are difficult to control despite multiple medications.

What it looks like

1. Focus on the symptoms that matter most

* Reduce seizure frequency, duration, and distress.

* Prevent prolonged or repeated seizures when possible.

* Reduce anxiety, pain, agitation, and the physical burden of seizures.

* Accept that occasional seizures may continue if aggressive treatment would cause more harm than benefit.

2. Choose medications based on comfort and practicality

Instead of adding multiple drugs with significant side effects, clinicians may favor medications that are:

* Easy to administer

* Sedating when sedation is desirable

* Available through non-oral routes if swallowing becomes difficult

* Less likely to cause troublesome adverse effects

3. Have a rescue plan

Families and caregivers are often given a very specific plan for what to do if a seizure occurs—for example:

* When to administer rescue medication

* When to repeat it

* When to call hospice/medical staf

* When hospitalization is or isn’t consistent with the person’s goals

4. Avoid burdensome interventions when appropriate

Depending on the person’s goals, the team may decide against things such as repeated emergency-department visits, extensive diagnostic testing, IV medications, or ICU admission if these interventions are unlikely to improve meaningful quality of life.

If seizures become refractory—meaning they continue despite appropriate treatment—the goal can shift even more strongly toward comfort. Medication may intentionally cause substantial harm..

An important distinction

A palliative approach doesn’t mean “don’t treat the seizures.” It means:

Treat the seizures according to the person’s goals and overall condition, balancing seizure control against medication burden, alertness, cognition, and quality of life.

For someone with a lifelong neurological condition such as Dup15q, for example, a palliative seizure-management philosophy could potentially be appropriate even when the person is not dying—particularly if seizures are medically refractory and treatment decisions need to prioritize comfort and function.

Second ER Visit of the Year

 

Just when it seemed we are all clear of medical emergencies for the year, I received a call from the school nurse saying that Luki was transported to the hospital emergency room after gashing his face from a seizure fall.  We are fortunate that the hospital is right behind his school campus and he was promptly taken by the paramedics via a gurney to the ER department.  His teacher said he fell from being seated to the ground after a drop attack.  His 1:1 aide was on her lunch break so he was being supervised by another classroom aide who was clueless about the nature of how quickly and dangerous the seizures can be.  His teacher said the guy was pretty shaken up afterwards and I feel very bad for him because this has also happened to me even with being very vigilant.  

I found Luki sleeping, post ictal, on a gurney in the ER hallway with blood stains all over his shirt, shorts and shoes and found dried blood streaking down his face.  The above picture to the left is taken after I cleaned him up.  It was like watching a scene from a horror show.  The paramedics and the school principal accompanied him as they waited for my arrival.  A very young resident explained she would do the sutures but seemed nervous as Luki would not sit still and would use his hands to resist her or attempt to snatch her tools out of her hands. I swaddled him up with a sheet but at 15, he is much stronger than when these incidents happened at younger years when it was so much easier to restrain him.  I suggested a little bit of ketamine to knock him out as this was done in another ER visit to get sutured for another wound.  She put the orders, I signed all the consents and liability agreements, the respiratory technician was called to bedside and the resident doctor was able to suture his wound in 15 minutes just in time for the medication to wear off and for him to gain back consciousness. I sighed a breath of relief, and the doctor gave me a nervous laugh as if I were echoing her same sentiment.  I remember we went through a similar ordeal for him on the other eyebrow for which he didn't receive any sutures and now half his eyebrow permanently grows no hair. We waited for the ketamine to wear off, monitored him for any signs of vomiting which could be from the sedation or a brain bleed from the fall.  Once cleared, I took him home with him being back to his cheerful good mood.  

As a parent, it can be quite lonely to be going through something like this but wouldn't you believe it   this webinar (see below) came to me exactly when I needed some reassurance that I am not failing my son by not having his seizures completely under control.  It is also comforting to belong to a community of parents who get what I am going through.  These moms speak my language, echo my sentiments regarding seizures, antiseizure medications and quality of life. The most helpful advice these moms shared is to not be so hard on ourselves when our kids' seizures are not under control because sometimes, I feel like I am failing him when these emergent situations arise.

.

  While living in the unknown and the mystery this syndrome presents and when no cure or proper treatment is in sight, it is so good to find a community who can sit with me in the uncertainty this life presents. Their presence makes all the difference. 

Caregiver’s Guide to Seizure Medications Part 1 - YouTube