As I set out to write a story of Miranda, the first thing that pops into my mind is old familiar song lyrics, “how do you catch a cloud and pin it down?” She is amazing, challenging, magical, and profoundly an individual. However all of these traits might not be obvious at first glance. The first thing a new friend might notice is her wheel chair, then her tube to her tummy and then maybe the line that is always monitoring her pulse and oxygen saturations. But before the lines, they might notice that she has a hard time looking at them, and that her hands rarely relax in her lap or any where unless she hold something upon which she can chew. She relaxes more that way.
Miranda has a rare genetic condition, Bohring-Opitz syndrome. When she was born we were told all kinds of bizarre things in an attempt for someone to make sense of her and how she medically presented. All the known genetic tests were done and all came up negative. This was 2004. A lot has been learned since then.
I remember when I was finally able to see her. The cutest little dark hair covered mini-being; so much hair on her head and dark fuzz all over her body, all curled into her 16 inch self. Such tiny hands and lovely little face on a lovely little head, big huge eyes and a cleft in her lip all the way to her nose – my tiny little mouse.
I wanted her to be breast fed with all of my heart, but she wasn’t able to form suction, but worse than that, she didn’t even seem interested. She seemed to have no interest in eating or survival. I was shocked and scared. I kept asking her, “Why don’t you want to live?” But I pumped anyway, we had an NG tube placed and we learned how to use it. I smile at the memory. Carlos was always better at placing it than I was, but we were a great team. I would pump, and he would give to her, her feedings every 2 hours around the clock and we had almost enough milk to start our own dairy.
Miranda had tough beginning and because no one knew what to expect, we were winging it. She presented her first tonic clonic seizures at 4 months. We were told that she could have her cleft lip repaired when she reached 11 pounds. That didn’t happen until she was 6 months old, and she was barely keeping there. We struggled so much with keeping food inside of her in spite of all her medicines. But we didn’t know if she could hear or see. We hardly knew if she was breathing regularly. Miranda wore an apnea monitor, but we were so clueless. Testing positive for Laryngomalacia, helped the birth to three folks and we finally had some path to follow. When her cleft lip was repaired, we actually had a hearing test, but her ears were filled with goopy discharge and they didn’t’ even know if the results were true.
Miranda was in the hospital continually because of cyclic vomiting. She would vomit until her esophagus bled. These were some of the most trying days of my life – Seizures, vomiting, despondent, a missing girl, almost, but she loved her Raggedy-Ann doll, who we call Roganet. That little doll became Miranda’s focus, and brought so much delight and has attended almost every surgery.
Miranda was put into the hospital at 20 months old weighing in at a hefty 17 lbs. with aspiration pneumonia. She had very low oxygen levels and was struggling to breathe. For 6 weeks we lived in the PICU and she was intubated with an endotracheal tube, living off a machine, sedated. We read to her Ramona the Brave and The Little Prince. She failed extubation 3 times and one time coded blue and needed resuscitation. In all of my life , I have never been so scared. After a successful Nissen Fundoplication, a surgery to stop reflux, she finally started to heal. After a two-month stay, we were finally on our way home.
She turned 2 years old shortly after her release from what I began to think of as prison. We were told most children like her don’t make it to 2.
We celebrated that victory with a cupcake decorating party. Side note – I was a graduate student in a cross-disciplinary art program; most of our guests were graduate art students, that cupcake play became quite competitive. But I have to say maybe that was a precedent. Today, Miranda’s favorite desert remains – a chocolate cupcake.
Miranda is now approaching 11 years old. Focusing too much on her earliest years seems to reduce her to the medical trauma and difficulty. However, although that was her last long hospital stay, it wasn’t the end of it.
As I write along, I feel the need to pause to say that while we were in the hospital so often, I was also working on my Masters of Fine Arts at the University of Wisconsin-Madison. I am grateful to my faculty and peers at UW-Madison for their support of my family. Without that I may not have the career I do now.
I imagine as a reader you are curious; did we know anything about her before she was born? Actually, we didn’t know that anything was different. One reason was because the known tests revealed nothing, and the second was that the doctor never viewed the last ultra-sound we had at 37 weeks. He admitted to that personally after learning of her difficult birth, one that left her blue and needing resuscitation. We may have known something, but other than giving us better preparation, it would have changed nothing. So we will never actually know if the difficult birth played any role in who she is, but we are finally relieved to have a diagnosis.
Quite a few days later and I am back to writing again. I am thinking that I want to talk about her growth. Miranda is little for her age. She has had a long growth struggle and was tested early to see if she would benefit from Growth Hormone shots. The testing resulted in a diagnosis of hypothyroidism, which actually seemed a relief in comparison to the Growth Hormone course. She is 11 years old and has a height that is approximately 44 inches. We don ‘t know for sure because sometimes they measure her joint to joint and sometimes they try to stretch her on a bed in the examining room, measuring by drawing a line at the heal and a line at the head. She is so tight muscularly that it’s never quite accurate. She seems to shrink and grow arbitrarily. However, the general idea is that she is small for her age. She weighs about 49.5 lbs. and has stayed at that weight for about 3 years. It seems like boring statistics, but it is significant to us on many levels. On one level, we have to transfer her and mover her around in everything that she does. Her small size is great for that and a blessing in disguise. But almost 50 lbs is not taken lightly; so, I keep up a steady course with a personal trainer knowing that I will always need to be her physical support. On another level, her growth was actually compromised by a physician oversight. Miranda achieved early puberty. Although I continually brought signs to her endocrinologist’s attention, no blood tests were ever performed and I didn’t have enough information to demand them. Her early signs were dismissed as insignificant; thus, her body was early exposed to estrogen allowing her bones to close and growth to stop, a very layman interpretation. But the results are the same. Once her menstrual cycle began, they predicted a maximum growth of 3 inches…maybe Miranda will be 47” one day? She will be a cute little lady what ever her height, but I want to raise awareness that a parent should always push for their gut intuition. I knew she was changing, but I was treated as the girl who cried wolf. Afterwards, the doctor had the good grace to admit that she could have known if even on one of my alarm visits resulted in a given blood test; the information would have been there. The course might have been changed.
So about Miranda again- what is her life like everyday? Well, she is the example of ultimate forbearance and adaptability; that is her personality and our joy. Miranda wakes up and sends slight sound signals that I have noticed few can hear. But myself and her papa hear the changes in her sounds, the little glitches and exasperated grunts that say,” I am awake and I need to pee!” We rush into the room, turn off the food that is running into her through her g-tube, and turn off the pulse-oximeter that is monitoring her heart rate and oxygen levels, all in a singular important effort – get her to the potty on time! She likes to potty on the toilet and that is such an important way to start the day. We have to carry her there. And her smile at success is worth the whole scenario. How many of us take for granted that first ‘greet the morning’ pee? That is my little girl and almost an easy way for someone outside to see.
She loves food, although she gets most of her nutrition through a gastric tube and a feeding pump. One of her greatest ways to teach us has been through her relationship with food. My word choice here is important. She teaches us. Miranda enjoys learning, and she is greatly motivated by food; so Miranda will more easily make a choice if food is the goal. What eating with her teaches us is that she can make a lot of choices and how to approach other problems. So we model that. We have started to learn with Miranda how she can paint, write, answer learning questions, solve patterns, all based on how willing she is to make choices at eating time with her hand to point, touch a button, eye gaze or even grabbing. Releasing the food to chew has helped her release a ball to throw. It is amazing and so gratifying to witness all this connectedness and watch in adoration!
A dear friend encouraged me to speak to the fact that Carlos and I raised Miranda through her first 10 years without being surrounded by family or other interactive parents. We have had no models. We lived in Wisconsin away from any family, although we have had supportive visits. Because of our professions, we had few direct interactions with families with similar aged children. He works remotely and many of my artist friends did not choose to have children or those that did have children, well, it wasn’t part of our discussion. I feel that I can address that directly – my family with kiddos around Miranda’s age have always seemed reluctant to talk about their typically developing children and their own raising children issues. I have often felt it came from a place of ‘how can they complain’…But I truly believe that hearing their struggles would help normalize mine. Any way, we don’t know anything about raising kids, talking about raising kids or even the fact that it’s okay to say kid or kiddo….
Until now, we have not had any support groups or any one to pave the way. We have been unwittingly pioneering, learning about parenting and learning about Miranda as we go. .
This is now becoming something journalistic in style, without intention. Today I started thinking that maybe it would be interesting and helpful to make a count of Miranda’s procedures under anesthesia. I have a memory long ago of my dad’s wife being so upset that her new adopted daughter would have to undergo surgery for ear tubes; so, when Miranda’s first surgery was eminent I was pretty worried. So, here is a list of what I can remember has been performed under anesthesia (each bullet is one anesthesia):
• ABR hearing test, cleft lip repair, bilateral myringotomy
• g-tube placement, muscle biopsy for genetic testing, MRI
• Spinal surgery to release tethered spinal cord
• Eye biopsy for genetic testing
• 2nd adenoidectomy with new myringotomy
• G-J tube placement
• G-J tube removal, replace g-tube.
• Nissen fundoplication
• Strabismus surgery right eye
• Another bilateral myringotomy
• Removal of extra teeth and 9 baby teeth that needed to make room.
• Right ear drum fluid removal and repair
• Ear cleaning, new hearing aid molds and ABR
• Bilateral strabismus
• Spinal MRI
I may have forgotten a few. Along with these remembered, Miranda has had a number of scopes with contrast to test gastric motility, swallow studies, regular ultra sounds on her kidneys and bladder, CMG Bladder tests, and has had her ears vacuumed too many times for her to be happy.
I feel compelled to list these because one phrase I hear repeated in my head is ‘will it ever end and will I ever stopped being surprised?’ I have finally accepted that we will have a future of tests ahead. New on the horizon is another left ear fluid removal and myringotomy. But I am pretty sure that I will always be surprised.
Why? Because life moves along and things get a rhythm, day-to-day routine takes over; Miranda goes to school, therapies, attends a Birthday Party….
Oh, Birthday Parties. These have been difficult. Miranda attended school in a general education classroom. Her classmates adored her. They invited her to their Birthday parties. Where do Wisconsin children have Birthday parties? At Gyms, at Play jumping rooms: all things that Miranda cannot do. I have actually tried to carry her up inflated slides so that she can share the experience. I have actually left a party crying. As much as I want her to be able to participate, sometimes I am not the strong one. And I have no idea how to talk to these parents who are so happy at that moment, so happy that we came, and I am falling apart.
But somehow they never mention that they noticed, I hid it well, or they were obtuse or perhaps gracious.
As I have mentioned before, Miranda is continually monitored for oxygen and heart rate; she also sleeps with a bi-pap. It has been our choice to not have nursing at night. With that choice means that we have decided that one of us always sleeps with her. Perhaps other parents manage this differently, with monitors in their rooms, I don’t know. We feel best this way. Choosing to be with Miranda at night means a different way to have a relationship, yet it has afforded us privacy at night. It does mean that sleep is disrupted often. Changing her position half way through the night because she doesn’t move her self, adjusting the bi-pap mask, responding to oxygen changes and adding it when needed, taking her to the bathroom. That is a part of our life. We take turns.
Equipment. Perhaps to some parents, that means baseball bats and soccer balls, uniforms and sweaty teammates in their cars. What does it mean to us? Wheelchairs, standers, knee immobilizers, AFO’s, TLSO’s , wedges, inflatable peanuts, bath chairs, potty seats, Hoyer lifts, accessible vehicles, communication buttons, hearing aids, glasses, syringes, feeding pumps, oxygen pumps, pulse-oximeters, couching machine, suction machine, bi-pap and medicine. Although I guess meds are not equipment. With all this equipment comes therapy – PT, OT, and speech and feeding. With all the medicine comes a watchful attentive eye. It goes hand in hand. But no parent lives with out a schedule. Ours is just a little more intense.
So as I conclude this entry and bring it to the present, what are we watching now? What is on the top of Miranda’s health agenda? We struggle with things that most mammals take for granted, things that happen easily and automatically for most – Eating, peeing, pooping and breathing. At first it may seem a joke. It is not. Every day we have to help Miranda pass a bowel movement, everyday we are counting on her voiding and hoping that we do not have to give her a catheter, everyday we have to provide her with nutrition mechanically, enough to sustain her because she is not capable on her own, and every day we have to give her enough time on a machine to make sure that she breathes through the night.
We do all we can to supply and support these needs, we are blessed with nurses and providers that help us achieve these goals.
But as parents, grandparents, friends, uncles and aunts know – One thing that she needs to count on, one need shared by every 11 year old in the world is LOVE and lots of it! I am so happy that Miranda is blessed with this in abundance!