The Doctor is In….
One thing you can’t really prepare for in the life of a special needs parent is the sheer amount of education you receive. You will be surprised how much you retain, and how much you forget while providing care for your child. I spoke in previous blogs about how we live double lives as special needs parents. We play the role of Dad/Mom but also caregiver. I hope to be able to provide some insight, using my own experiences, and maybe along the way a couple tips when it comes to dealing with the real medical professionals.
When we first got the news that Hannah had some severe developmental issues, we instantly went into research mode. We did the worst thing imaginable at that point and googled everything. Information can be scary enough, but the open access online can be jarring. Overwhelmed is another good way to describe it. But ultimately, we only knew what the researchers knew, or what we were told by doctors. None of our research was structured or focused, and we were grasping at straws to try to understand what we were in for. It was a big guessing game. Images and ultrasound can only tell you so much. A part of me at that point (always being Mr. Positivity) was hoping for the mildest issues. Maybe like shelly, she would just have some cosmetic issues. Things we could deal with through surgery, but we would still have our little girl, who developed like most children. Looking back now, that was very wishful thinking considering what the doctors had tried to prepare us for. Maybe part of that was trying to keep Shelly calm, and looking forward rather than spending our time dreading the inevitable. Even if our research was a fool’s errand, or just a distraction from the very real stresses to come, it worked out in our favor.
When Hannah was born we realized how intense this journey was going to become. Of course, my positive mindset went straight to the worst case scenario as soon as the excitement from the physical birth went away. She was having difficulty breathing, and keeping oxygen saturations up. she was having long lasting seizures or what we came to know as “clusters.” I know Shelly and myself were just counting down the time we had with her. We were crushed. All our preparation and research hadn’t (and couldn’t) prepare us for what we were experiencing.
I remember holding Hannah sitting in the NICU of our local hospital. She was tiny, with big beautiful blue/grey eyes. You could see her struggling, feel her trembling with the seizures that racked her body. I felt helpless. All we could do was hold her. That’s what we did. I had made that promise, we see this through to the end, we keep fighting as long as she does.
That wait for our medical transport was excruciating. I remember being up with Shelly in her recovery room, and the neonatologist came in to give us their assessment. “She’s having seizures” in my mind I’m thinking “No shit, Sherlock” he continued “She has global development issues” again I’m screaming in my head to give us some news we don’t know yet. “We think she has trisomy 13” I about fell out of my chair. We both looked at him and said “no, she has Holoprosencephaly” he repeated “we feel she has trisomy 13… given our current assessment” This was a key learning from me from the very first hours of Hannah’s life…The doctors don’t know jack.
If you are a medical professional reading this, please know I have the utmost respect for your occupation and what you do on a daily basis for your patients. Please don’t take offense to the contents of these next paragraphs.
We have found through our travels…many specialists, many doctors, many hospitals…The doctors only know what they know, or what they have experienced. It’s a plain and simple truth. When we think about medical school, and the rote memorization we expect of our doctors, we never really consider that most of what they know came from either a textbook/journal, or from clinical experience…. practice, practice, practice. Maybe that’s why we “practice” medicine…we never really have mastered it in any appreciable way, it’s constantly evolving. When it comes to a living child with Holoprosencephaly, you become hard pressed to find a doctor that has more than a textbook paragraph of information on the subject.
Most conversations Shelly and I have, start with us stating “she has Holoprosencephaly,” followed by a long searching gaze by said doctor and the reply “yes, I’ve heard of it, but never have I seen it before.” To be fair, on the specialist’s side, they treat symptomatically what is present in front of them. She has an underlying diagnosis of HPE, however that is just the “condition” that causes the symptoms which we are treating. So, our neurologist is just treating the seizures, our endocrine is just monitoring her hormone and blood levels, our pediatrician is just treating her colds, infections, and providing a baseline standard of her care. We can’t expect them to be subject matter experts on her entire condition. Though we do use her as a teaching aid for them, so now they have experience. Maybe another family can benefit from that (I always find that silver lining)
Something else became apparent during our last stay at the hospital (our first and only so far). It’s not about memorization and recall as a doctor, it is about where and how quickly you can find GOOD information. Arnold Palmer children’s hospital is the only level 4 trauma center in Central Florida where we live. As such, they have the best teams of doctors that deal with Hanna’s condition. But above all else it is a teaching hospital. When I describe a teaching hospital, think about County General from the TV show “ER”: it’s a bunch of students “getting their hands dirty.” They do rounds with an attending each morning, and then you see teams of students and residents throughout your days, sometimes one assigned to you, sometimes whoever is available. But what we learned in the daily rounds is this. The attending and residents quizzed the students, and while some info was expected knowledge such as blood level ranges, and what they contribute to. A lot of information was quickly looked up on their phones, or they reported back the next day after spending some time reading in the lounge. It was fascinating that like many aspects of our digital age, medicine is becoming about knowing how to find the information, versus knowing it off the top of your head. So, this goes back to my original thought, you can’t expect the doctors to know more than a cursory understanding of a condition unless it’s their specialty, to a med student they need not memorize everything.
So I guess my biggest advice when going into the hospital is: be patient with the doctors as they come to understand your child. With advances in medicine and the technology available, a condition like Hannah’s that was all but a 100% mortality rate, is now seeing children surviving, and now they are presenting clinically. So, it’s going to be a learning curve. I’ve found comfort knowing there are other HPE families out there, and now our doctors seem to be more and more aware of it.
So back to the story… Once we were discharged from our local hospital and taken to the NICU at Winnie Palmer (Sister hospital to Arnold Palmer) our education really began. The neonatologist started addressing her seizures, ordered an MRI, EEG, blood labs and immediately began Hannah on the common seizure protocol with medicines. Things were moving quickly and we didn’t understand all the jargon and acronym soup that was being thrown at us. So we used our “down time” to start our research over. We finally had some focus, we finally had a better understanding of what we needed to prepare for.
So through the MRI they confirmed her structural brain abnormality. Confirmed as HPE and a severe semilobar to mild alobar variant. So her brain has a small degree of separation but it did not go as far as to completely divide, or substantially affect the fusing of her lower brain structures like her Thalamus, and Hypothalamus. They also informed us of what they called “Agenesis (absence) of the corpus callosum” through our reading that is the midline structure that acts as a neural highway from left to right side of the brain. It organizes our electrical activity into highway like “lanes” which send signals to the right places, to make our body function as it should. Surprisingly this condition or absence of this structure (partial or full) has been found in autistic children and some other developmental disorders. Makes a lot of sense. Fine motor skills, thought processes etc need to be organized, and if you don’t have this structure to act as guide-rails, the signals go crazy routes to try and compensate. So while a normal corpus callosum might resemble I-95 going in nice neat parallel lines, Hannah’s brain structure is more like the “NEW ultimate I-4”….it’s a mess.
Next up was the EEG, this is a study of brain electrical activity from several quadrants of the brain. Probes are stuck to predetermined points on the scalp to measure certain areas of the brain. Hannah put on quite a show for them. Through this we learned how her seizures originated, the types of seizures she experiences, the duration of the seizures, and what her recovery baseline was. Most of her seizures originated in the temporal lobe, and then migrated to the other sections of the brain almost like a ripple effect. She was having several different types of seizures: complex, focal, febrile (as she overheated from the other types) tonic/clonic, atonic drop, absence, and petit/grand mal. She ranged in the 100s of seizures per day. Sometimes clusters lasted for 30-60 minutes as we struggled to find any control with medicines. Eventually the neurologists who read her EEG said her epilepsy was likely intractable, meaning there was no way to really control them from a pharmacological standpoint. Again I’m using big words. All learned while sitting in the hospital worrying…at least it was time well spent.
We were in hospital with Hannah for about two weeks after her birth. In this time we read and we learned about her care. We learned how to bathe her, how to administer her medicines, we witnessed and learned through several EEG sessions, learned how to feed her and how to place an NG/OG feeding tube and check placement with a stethoscope. Crash course in nursing for sure.
Hannah also started experiencing some severe side effects of what we learned were some negative effects of barbiturates and AED (Anti-epileptic drugs) . These drugs are severe system depressants, and at higher doses with a very small child can make breathing difficult. Hannah was now on full time oxygen. It was scary. We were taught the proper protocols to take her home on oxygen. I think the team of doctors treating us all assumed Hannah wasn’t going to survive long. I remember that room full of her doctors telling us we needed to prepare ourselves, and the palliative care doctor having us sign her DNR and telling us “maybe she will pass when we sleep and it will be peaceful.” Not the words you want to hear as a parent. But it did solidify my opinion that the Doctors don’t know shit.
Once Hannah was home, our education was put to the test, we were on our own…literally. How much of her care routine did we commit to memory? How did we cope?
My wife is the queen of organization. Remember she is a teacher through and through. She thrives in planners, notebooks, journaling, etc. so when it came to Hannah there was a LIST, a CHART, a CALENDAR. It was the only way to keep track of everything. For new special needs parents, take this advice…. RECORD EVERYTHING. Write it down, keep it organized. Bring med lists, and a brief history to every doctor. You will repeat your story a million times to a million doctors, it gets easier, but in the early days make it easy by having it with you. So, as we learned, we updated our documents. Our routine became just that…routine.
Because Hannah was discharged under Hospice/ palliative care we really didn’t have a lot of treatment options. We were allowed to see a pediatrician, as they just took care of her comfort. We couldn’t get therapy; we couldn’t see specialists. We couldn’t seek treatment. They were long ..sleepless nights in the beginning. I remember like all parents being up at 2 in the morning as my wife slept, taking care of our little girl, trying to control her seizures. Fighting off her febrile seizures by soaking rags to cool her little body off as she exhausted herself from seizure after seizure. It was torture for her, but also for us as well.
Finally, we realized that the palliative care, and hospice treatment of Hannah was not doing her any favors. She was suffering more with it. We needed treatment, not waiting for her to die. We made the decision together as her parents that if Hannah was fighting, we would fight too. I remember it was just about eight weeks into her life that we finally started searching for our medical team that would work with Hannah, not just for comfort, but to give her the best shot. A few days after we had all our doctors set up, I remembered the DNR order we had posted on our fridge. I tore it down, I threw it in the BBQ grill, and I set it on fire. We were going to fight if Hannah was able to! That was the best tasting steak I ever cooked.
With new doctors came more education. We learned the specialties. We both attended appointments together, always as a care team. The doctors appreciated our curiosity above all. We wanted to help in caring for Hannah, we weren’t just expecting them to solve it alone for us. We were organized, we quickly learned, and we learned how to speak their language. We found doctors are more comfortable if you are speaking to them as a colleague, not just a service provider. We gave them our opinions and asked for theirs. It was a very give and take relationship. But it wasn’t always smooth.
I found the doctors (especially the male ones) felt more comfortable talking to me, versus my wife. We would both be in a room, but they would speak directly to me. They would acknowledge her only when she mentioned something but would go right back to addressing me. Shelly is a very independent woman, she speaks for herself, and she isn’t afraid to disagree. I think this behavior ticked her off more than anything. But I can’t help but feel some of the blame. As I stated above, doctors are comfortable when talking to colleagues. Through my self education I use a similar language when talking with them. So maybe how I talk might have just made them more comfortable talking with me. That and my tone is always very even and unassuming, and Shelly tends to get worked up especially when she doesn’t understand what they are trying to say with all their jargon. This is something I think medical professionals need to work on as a whole. Some call it “bedside manner” but really it’s just communication. And we’ve had some doozies for sure. More on that later.
So DNR is gone, we have our doctors now, and we are navigating this life. For the last 8 years this became our role, as it continues to be today. Every day we learn something new. We connect with more experiences, and encounter different obstacles. We have become Neurologists, Endocrinologists, Pulmonologists, Respiratory therapists, and nurses all rolled into one. While we don’t hold the needed degrees, we have found our education has helped immensely in this. Hannah owes her life to this. We engaged and we discovered. We have seen too many families fail in this and know the outcomes that happen when you give up, or when you don’t learn, or practice what you should.
The biggest role you play as a special needs parent is ADVOCATE. Our kids are non-verbal we need to speak for them. The doctors do not walk our life, they do not see the day to day. They treat by textbook. We have gone through countless doctors finding the right ones that would work with us in the way we needed. Many families have. You have a say in your child’s care. You also have a responsibility to be active in that care. I know several dads that have had to get aggressive with doctors because they disagreed with our course of treatment or simply wouldn’t listen to our story and what we were telling them.
Our hospital stay recently put this to the test. Hannah had never had sodium issues before her bought with pancreatitis and subsequent saline hydration. But she was extremely high (180) when the high(dangerous) range is 140 on a balanced scale of 130-140. Dropping it too fast can lead to severe edema (swelling) in her brain and could ultimately kill her if we weren’t careful. It took weeks for them to try and figure this out. I remember one night, as we were learning her trends, we realized that the residents and attending were just making changes too quickly for Hannah’s body to catch up. Every lab value that came back Q4 hours was met with an adjustment. And we were swinging high and low. Poor girl was retaining water and edema was apparent in her arms and legs. And nobody was coordinating. I finally had enough and called in the overnight attending. I told him they were going to knock off this knee jerk reaction and wait at least two tests before adjusting anymore. They weren’t allowing her system to work through. I remember the next morning the chief attending was going around at rounds, and she pulled me aside after they wrapped up with our room and told me she heard I had talked to the on-call attending the night before. She gave me kudos for seeing what they didn’t. She repeated that sometimes the parents do know what’s best and she had spoken with her residents that morning about listening to the parents, especially those that are seasoned special needs parents like we are. Chances are we know something they don’t or that they have overlooked. We know her baseline…they don’t…they treat textbook, and they treat clinically.
You. Have. A. Voice…..USE IT!
I hope through sharing this side of our story you can see how valuable knowledge is. How being present and involved in your child’s care is beneficial for your child, but also you as the parent. We do share some unique struggles being heard by those whose care we are in. but be empowered to use that voice. To question respectfully, but firmly. For our kids that don’t have a voice, we are all they have. In between our appointments we are the doctors, we are the caregivers, we are the parents.