February 3, 2012
An update from Natalie...
Tim and I both work in professions that involve betrayal. As a physical therapist, Tim witnesses betrayals of the body. As someone trained in clinical psychology, I witness betrayals of the mind. Because of the complex intersections between body and mind, we also bear witness to assaults that begin one way (say an accident) deeply altering the other. Perhaps these professional experiences gave us a slight edge when Kyra’s body underwent an ultimate betrayal, a loss of the ability to sit up, let alone stand.
On June 1st, 2010, Kyra contracted a nasty sinus infection (I remember this date vividly because the day prior was Memorial Day and that day was one of those splendid, perfect days – Kyra and I spent that morning walking the 5K Bolder Boulder with her friends and the rest of the day was spent with family and some of our most cherished friends, Rick and Maria, who were visiting from New York). There was something about that infection (or something about that infection plus the repeated illnesses she had experienced during that school year, or something about that illness plus a genetic predisposition) that sent her tumbling down a rabbit hole. Literally and figuratively, her world went topsy-turvy and she could not right it. Or, more specifically, upright it. We watched Kyra struggle to sit up for even a minute at a time. We also watched an illness that should have cleared up within a week, take a prolonged grip on her and then morph into a set of new, confounding symptoms.
What on earth could cause a child, eager to set out on her summer adventures, such difficulty? At least part of the explanation was hidden in plain sight. Actually, in plain sound. When we first began bringing Kyra to medical appointments, the nurses/doctors would often comment on her rapid heart rate. We attributed it to nervousness. In retrospect, that attribution was mostly misplaced. Although Kyra was sometimes nervous (if she knew there was going to be something like a blood draw), she often was quite calm, all things considering. If anything, she looked like a kid who was seeing the doctor because of the flu. She didn’t have the energy to be nervous. It took the right doctor to interpret that rapid heart rate in a different light. This is the doctor who assessed Kyra for Postural Orthostatic Tachycardia Syndrome (POTS). POTS involves a dysregulation of the autonomic nervous system. The autonomic nervous system regulates things like blood pressure and heart rate, things that are assumed to be mostly involuntary (Wikipedia has a pretty good description of POTS for anyone who cares to learn more). The more severe forms of POTS can be disabling, life altering. It is more than safe to say that Kyra’s has been disabling and life altering. Prior to our arrival at Mayo, she had been in bed for 17 months and in the hospital for four of those months.
Syndromes are funny things. The syndrome label often is used when doctors see a pattern of symptoms across patients but really don’t know what is going on. The label is a type of “best guess” to get the medical and research balls rolling. As a diagnosis, POTS has only been on the block for about a decade and everyone is still trying to figure out what type of neighbor it is. Who knows if this diagnostic category will hold up over time? It may get spliced into subcategories. It may be subsumed under a different disease process (or processes). Because POTS is a fairly recent discovery and is relatively rare, it was crucial that we find doctors who were familiar with POTS and the cluster of symptoms that often accompany it. Mayo (in Rochester, MN) is one of the only places that has seen many children (and adults) with POTS so we were very relieved when we found out in December that they would expedite Kyra’s case (there were only a handful of hospitals that we had to choose from and all had wait lists of over a year).
Here is what we have learned since our arrival in mid-December (and from our own research): the onset of POTS typically follows an infection – in MN they usually see it with mono and Lyme but West Nile can be a culprit as well (West Nile is known for its neuroinvasiveness); POTS is related to connective tissue disease processes (we have a history of these in our families); at least 1/3 of people who receive a diagnosis of chronic fatigue syndrome (which has been renamed myalgic encephalomyelitis) also meet criteria for POTS; hormones appear to play a role in the development of POTS; POTS is associated with low iron stores and anemia; many children with POTS have GI symptoms as well; some new research in an adult population suggests POTS may be an autoimmune disorder, possibly of the cardiac system. The very good news is that many kids with POTS outgrow it by early adulthood. The research is still quite fragmented but there is enough of it to start assembling a scientific story of these kids.
Right before departing for Mayo, we met with a metabolic specialist at Children’s hospital in Denver and his assessment was that Kyra most likely has a disease process that manifests neuromuscularly. Interestingly, I just ran across a disease in certain breeds of dogs called Exercise Induced Collapse – it appears to be a metabolic neuromuscular disease and has many of the same properties as POTS/Myalgic Encephalomyelitis. The University of Minnesota recently discovered the gene responsible for it. It is highly unlikely that they will discover a single gene for POTS but they certainly may find genetic contributions to it. I found out about Exercise Induced Collapse because we are thinking of getting a dog (when our lives settle down) and I have been researching potential health complications for various breeds. One of our top contenders, a boykin, is prone to EIC. How crazy is it that out of all the breeds we could select from, we chose one that has some of the same vulnerabilities as Kyra?! Sorry to say, that dog is off our list, at least for the time being.
So, here we are at Mayo. We have been here since the 15th of December and will remain for another two to five weeks. We have had a very positive experience and our primary doctor has assembled a strong team to help Kyra regain her health. The first step in her care was to assess for metabolic causes of her low energy. Kyra’s ferritin (which indicates how well iron is stored in the body) and hemoglobin, (which carries oxygen) are both low so Kyra receives an iron infusion about once a month. She also has been on immunoglobulin treatments every three weeks since October and it appears that those have helped reduce her vomiting and also help protect her from various illnesses.
The next step was to begin intensive rehab, which includes Physical Therapy and Occupational Therapy and meeting with a psychologist who works with children with POTS who develop GI issues (she will help guide the reintroduction of food that will take place once Kyra is stronger). On a typical day, Kyra has PT and OT for three hours and then we continue to carry over the exercises for the rest of the day at home (which, for the time being, is the Ronald McDonald House – can’t even begin to say enough great things about this facility). The first month was very (very!) hard for her, as we knew it would be. Fundamentally, Kyra had to go to PT and OT each day with severe flu like symptoms, including nausea and vomiting. Just thinking about this almost makes me cry but Tim and I know this is one of the best ways to get a dysregulated body back to a place of health. To get through this, I keep reminding myself that we have been in hell so we can do hard. I also knew that it absolutely would pay off, which it has.
Going into this, we knew the two main challenges were going to be getting her upright after a year and a half of being bedridden and getting her eating again (she has not eaten anything for a year and currently is being fed through a PICC line). She basically has met the first challenge (she can sit mostly upright in a reclining wheelchair for several hours) and is working on the second (she can successfully swallow a few drops of water and we are now moving on to a few drops of smoothie). We absolutely needed a team of professionals who were very familiar with POTS to help with her recovery and we have been so appreciative of how dedicated and supportive they have been. Each day, we see a body and mind that is betraying Kyra less and less. Each day, Tim and I move toward resuming our roles of “just” being her parents.
Even more rewarding is watching Kyra and Tess resume their roles as sisters. Tess has been up here since we arrived and will stay for two more weeks (a huge thank you to Heatherwood Elementary for being so supportive of Tess’ sporadic attendance this year). Tim returned to work last week so I rely heavily on Tess to help me with Kyra (she loves this role and performs it better than many adults would). Mayo does not have valet parking so each morning I drop Tess and Kyra off at the main doors so that I can park. Tess brings Kyra up to the 16th floor and gets her checked in for her appointments. In Rochester (and specifically Mayo), a mother does not have to concern herself with safety as there are many people looking out for them as they make their way through the building (the doormen look forward to them coming and love to tease them). Plus, for those of you who know Tess, you just do not mess with her. That child’s street smarts are already way too well developed. We have been preparing for her teenage years since she was about three.
Both Tess and Kyra have completely made themselves at home at the Ronald McDonald House and participate in all of the events that are offered. The pet visits are some of our favorites. Tess was invited to be one of the child greeters at the annual Ronald McDonald House fundraiser Saturday night, which included purchasing a beautiful dress, getting hair and makeup done, and riding to the event in a limo – the limo is what sold Tess on the idea. We had such a great time watching her, and four other girls, get made up for the event. The other girls were/are patients at Mayo, and at least for the time being, their stories are ones of triumphs over tragedies. Kyra has no interest in physically partaking in anything that involves too much bling (she is a Boulder girl at heart) but she happily observed all that was involved in preparing a group of girls for a night out on the town.
Even in betrayal there is beauty.