Team Max Part Two

Max is diagnosed with a swallow disorder, and that disorder is causing asthma and a significant airway compromise. When Max sounds like he is getting sick, we have a list of instructions to follow to prevent him from developing aspiration pneumonia. Thick liquids, and a sick plan….

Max’s diagnosis is confusing to us. Dysphagia. A swallow disorder. We learn that anatomically Max has underdeveloped parts of his swallow mechanisms that are causing him sickness. When he swallows liquid, the muscles are not strong enough to bring all the liquid he drinks where it is supposed to go, therefore, we have to learn how to thicken liquids so they’re too thick to leak into his lungs (link provided at the end of the post for dysphagia resources).

In the hospital room, a feeding specialist came in with a Mary Poppins bag of “things”. She begins to take out gloves, packets of powder I’ve never seen before, a glass measuring cup, a fork, and a handful of syringes. Before she even begins, the tears are slowly and silently leaving my eyes.

We had absolutely no idea how to navigate this new territory we were being forced into. Max was breast milk through a bottle-fed baby. The instructions for him included a prescription thickening agent to be whisked into every single liquid he ingests. Those liquids include water, formula or milk and juice. All liquids. To thicken his liquids, the liquid must be at room temperature. How am I going to keep up with pumping and thickening and working and another two children?

I am absolutely overwhelmed and FURIOUS.

I am not angry with God or the Universe; I am not angry with the cards we have been dealt. I am angry that every step of this eight- month journey has been an uphill climb, and with every appointment and hospitalization the hill is getting bigger and bigger. Sometimes the tears were impossible to hide. I broke down in many hospital and exam rooms, allowing myself sixty seconds of feeling before jumping back in as care giver.

The only pharmacy that carried the thickener...Walgreens in Hartford across from the rear entrance to Hartford Hospital. Convenient? No freaking way and in a pre-covid world, there weren’t many shipping or delivery options.  To a Pyrex measuring cup add Max’s room temperature liquid, whisk in a packet or two or three of the thickening agent and use syringes to test the thickness. Set a timer on your phone and test the liquid. You cannot deviate from any of the products and directions given in the hospital. Run out of syringes because the numbers wear off from hand washing? Drive to Farmington for more.

If the liquid is not right, dump it out and try again. Keep in mind you need to do this just before Max eats and you’ll want to work on it before he gets upset. An eight-month-old is still VERY cranky when he is hungry. No pressure though.

At this time, Max’s care was becoming more than a full-time job. Weekly, in-person check-ins with feeding therapy (either in Farmington, Glastonbury, or Hartford), routine audiology exams that were timed with Max’s naps (a sleeping Max provided the most accurate data), occupational therapy at home, speech therapy and physical therapy at home. Carly is a full time at home toddler and Ian, and I are both trying to maintain full time jobs. No parent should ever be placed in the positions we were in. We were forced to work to afford the insurance and care for our son and yet our son required more care than we knew how to provide. How did we get through it? Looking back, I can see now that survival mode saved us. Holding our breath until that check clears, sleeping through the night when test results were finally returned. Every single day brought with it more challenges.

On a side note, as mentioned in part one of Max’s story, at this time of my career I have run into significant difficulty at work with regards to accommodations I require for pumping breast milk while at work. On top of keeping up with Max’s multiple therapy and doctor appointments, Carly deserves the most normal toddler experience as possible, and I now have meetings with the Department of Labor, Union representatives and the Commission on Human Rights and Opportunities. More on this to come.

As I mentioned in the first part of this story, the timeline is all over the place. We are progressing now into the nine-month stage. Fun fact, while “most” baby boys are growing towards toddler age, their testicles drop into their anatomically correct position. However, Maxwell’s did not. On a recommendation from the pediatrician, we have a consultation with a pediatric urologist. At this appointment we learn that Maxwell has an “orchiopexy” which is an undescended testicle. If left untouched, it could cause significant ramifications he will be forced to deal with as an adult. Because Maxwell is less than a year old, with significant breathing difficulties, no provider is jumping up and down to sedate him and perform any procedures. The dr asks us to coordinate with him should Max require another procedure or diagnostic evaluation that requires sedation. In the back of my head is the question, “how would I coordinate anything at the hospital?”

At some point, it is recommended that we switch to an otolaryngologist who specializes in cochlear implants; if we decide cochlear implants are the way to go for Max, we have an established relationship with the doctor who specializes in this area. For a few months Ian and I have been asking any provider who would listen about deafness, hearing loss, cochlear implants, and sign language. As I dive headfirst into learning about deaf culture, I/we make a decision. Max is our son, yes, however, the decision to mechanically alter his capability to hear is not our decision. I am not making a permanent decision an eight-month-old that could affect him when he is eighteen and is not life or death. We learn (and not from any of the doctors) that to successfully place cochlear implants, any natural hearing Max has will be eliminated. While the hearing tests are inconclusive and we learn Max’s hearing will likely progressively diminish, we have no idea when that could happen.

Max needs to have ear tubes inserted to drain his ears. Nothing can progress for him until this surgery is scheduled and performed. It is important and needs to happen sooner than later. We now have our “ticket” to sedation and sedation means answers.

Surgery scheduled.

In one hospital visit, Maxwell will be put under general anesthesia. He will have a complete spinal MRI, ear tube placement surgery, orchiopexy repair (testicle surgery), audiology testing and pulmonary will be in the OR to insert a camera into Max’s lungs to check on their developmental progress.

As Max’s procedural day winds down and he is brought to recovery, we have multiple meetings with multiple doctors. This is what we learn:

-Maxwell has “floppy lung” which is called bronchomalacia:

“Bronchomalacia is a congenital problem that arises from diminished cartilage support of the smaller airways (below the trachea, or windpipe).  The weakened cartilage usually collapses more easily during expiration and prolongs expiration or prevents expectoration and causes trapping of secretions. Symptoms of bronchomalacia vary but may include chronic cough, prolongation of lower respiratory tract infections, exercise intolerance, respiratory distress, apnea, recurrent pneumonia and recurrent bronchitis”(https://www.childrensnational.org/get-care/health-library/bronchomalacia)

-all of Maxwell’s issues are consistent with the left side of his body. Left ear, left lung, left testicle, left kidney, left, left, left...

-the MRI…We go back to neurosurgery to discuss the results. Maxwell has a tethered spinal cord. His cord is tethered way past where it should have ended and is wrapped up in an unknown mass. That mass has the possibility of being a tumor.

At that neurosurgery appointment our world changed. It was at that appointment, I realized I had a child with significant medical needs. At that appointment, I became someone different. Max requires serious surgery for his spine. Neurosurgery explains the severity without trying to worry us to the point of no return, but at this time it’s too late. We are deep in the throes of learning about spina bifida because Max has “occult spinal dysraphism” which is a form of spina bifida. The only way to diagnose whether or not the mass at the base of the spine is a tumor is in the operating room. Max’s future was wrapped up in the outcome of this surgery. Which is now scheduled for May. Before Max turns one, he will have major spinal surgery and there is nothing I can do about it.

At home, Ian and I are constantly on opposite pages about Max’s care. I am reluctant to leave him with anyone, yet we have to continue working. This is a constant struggle and when my parents offered for us to take over and rent their house so they could move to South Carolina, I jump at the opportunity to have familiarity and to raise the kids in Ellington, rather than East Hartford where we were.

Team Max is born from this inability for me to control or change anything about our situation. I am not interested in raising money; however, I am interested in giving Max the positive experience possible. The idea comes to me. Make a t-shirt that friends and family can wear on surgery day. Throughout the day people can send us selfies and photos of themselves wearing their supportive T-shirts. Photos and positive messages for Max…hope for Mom and Dad. From team Max, we received some of the most genuine, heartfelt, real and warm gestures. People we barely knew were dropping meals off at our house. Word spreads on social media and through Carly’s preschool and we receive messages, toys for Max, inspiration for us…various forms of comfort.

Surgery comes and Team Max is in full swing. The surgery took hours and when it was over, my baby was in the pediatric intensive care unit, on heavy medications to keep him comfortably sleeping…and still. The outcome of the surgery was both a relief and a devastating blow. While the mass at the base of Maxwell’s spine is most likely not a tumor, his spinal cord cannot be completely released from the mass. Tangled up into the mass along with his spinal cord are essential nerves for Max’s bladder and bowel control. If those nerve roots are severed to free the entire spinal cord, Max will lose his bowel and bladder control for the rest of his life.

At this point, we receive messages from well-intentioned friends and family. Often, we hear “how much more can this little guy take”, “why is it always something”? While we felt that and felt it in the depths of our soul, we could not think like that. In the waiting room on the third floor of the hospital, tucked back into a corner, I say to Ian, “one day we will look back on today and remember how scared we were…but we will survive it”. This became our mantra for years to follow.

Max recovers well in the hospital for the most part and I would like to mention here that I am responsible for thickening all of Max’s liquids in the hospital. Anything the child is going to consume has to be monitored. I don’t leave Max’s side. I can’t. I am consumed with worst case scenarios, how to build ramps for the house, how are we going to manage this, how am I going to manage more?

Spinal (and most major surgeries) surgery can often times shock the bladder. This is the case for Max, or at least that is what is suspected. The urology department checks in on him frequently. They come in with an ultrasound type machine that measures how much urine is left in Max’s bladder. They tell us that he has “residuals” that are not healthy; his bladder is not cooperating and not emptying appropriately. Shocking.

Enter catheterization instructions stage right. For Maxwell to go home, we have to learn how to properly empty his bladder with a catheter.

Multiple times a day.

I am a 911 dispatcher and former EMT, not a nurse; Ian is the general manager of a hockey store and not a nurse. What do we know about catheterization??  Not only about catheterizing our child, but doing it safely to prevent infection?? How are we going to manage this? How are we going to manage this with babysitting? I can’t see straight anymore.

The next few months are pure chaos. I am working in the evenings to be available for doctor’s appointments and therapy during the day. Ian, in retail, has the worst schedule in the history of the world. Ian’s Mom watches the kids from the time I have to leave, until Ian gets home, not much longer than 4-5 hours. Before getting myself ready for work and making dinner for me to take and for Ian and Carly to have, I have to pre-thicken ALL of anything Max could drink. This means making batches of liquid before we leave the house. This means nailing the recipe, otherwise, it all gets tossed and you have to start all over again. We are buying VERY expensive formula that has nothing but minerals and protein in it, because on top of all of this chaos, Max is not pooping like a typical baby either. During this time, I am taking my “dinner” break at work, provided I’m not working by myself and it’s not too busy to drive home as quickly as possible. I run in the house, squeeze the kids who are overjoyed to see me, scrub my hands, speed cath Max, get him all set for bed and then rush out while the kids scream and cry and beg me not to go…I then, drive back to work and count down the minutes until I can leave and go home again.  

Why didn’t I quit my job to take care of Max full time? Bills for sure, but also health insurance. I cannot discount the massive benefit that we were afforded by having municipal insurance. I would cry often for the families that did not have insurance. We had no choice but to work together to coordinate Max’s schedule based on our work schedules. We were chess pieces being moved around the board by whatever pawn we were to jump over next.

This is exhausting and not sustainable.

My in-laws showed up. They were with us in some capacity every day. Whether that was watching the kids so we could work, or taking care of Carly every time Max was hospitalized, they showed up. This is a significant “milestone” in the relationship I had with my parents. Aside from sending team Max t-shirt selfies from South Carolina, my parents were not present. They did not take any time from work or their new life and come to see me or Max…or Carly.  They did not show up and take over our laundry, dishes, or grocery shopping. They were not there to make us dinner and remind me to take care of myself.  Did my mom or dad show up and hug me and tell me that even though this was hard and awful, we would survive, and we were strong, loved and supported? I think you know the answer.

They sent me a portable coffee maker that my sister told them I wanted so I could make myself coffee when Max had hospital stays.

At the time, we did not think we had to explain a lot or much at all to Carly and Addy. Since hindsight is always 20/20, I see now that we should have explained a lot more than we did. Max’s sisters are resilient and incredible. Carly has significant anxiety these days which can be linked backwards to Max’s care.

It is the fall of 2018, we have a lull in diagnostics, and have been attending sign language classes at the American School for the Deaf. Even though we are not entirely sure Max is 100% deaf, the community at ASD welcomed us with beautiful and open arms. The people we were fortunate enough to meet at ASD became lifelong friends and the people who helped us through some of the most difficult times in our son’s life. In an effort to counterbalance what we could not control, we poured everything into what we could, such as learning ASL for our son. Carly and Addy attended sibling sign language classes as well (yes, we had to work ASL into our already INSANE schedule with Addy’s back and forth schedule, work and babysitting).  

As Max continues to participate in daily activity, his physical therapist and I recognize that something is not quite right. Max is an awkward walker; however, he seems to be dragging his foot when he walks. Holding out hope that this was temporary or a toddler glitch, we are back in neurosurgery… scheduling another sedated MRI.

Max’s MRI shows a pocket of fluid in his spinal cord that is prohibiting routine spinal fluid flow and function. This is causing Max to drag his leg to walk.

AAAAAAAAAAAAAAAHHHHHHHHHHHHHHHHHHHHHH!!!!!!!!!!!

He requires another surgery.

Six months after the first one.

In less than one year from the first. The neurosurgery team will drain the fluid pocket, which is called a syrinx, and place a stent in his spinal cord to prevent the fluid back up from happening again. Syringomyelia is what Max is diagnosed with. While in surgery, the team will try to release more of the cord that can be freed without compromising his bladder and bowel function.  After surgery one, we were not sure if Max would walk. Now, after surgery two, we are not sure if Max will be able to go to the bathroom on his own.

Part three to follow...

Resources:

Dysphagia : https://www.netmeds.com/health-library/post/dysphagia-causes-symptoms-and-treatment

Pediatric Broncomalacia: https://www.childrensnational.org/get-care/health-library/bronchomalacia