May 1, 2012
An update from Natalie....
Hello to all! It is nice to put words to “paper” again. Writing is usually therapeutic for me but one has to have energy to write and until recently, that just was not possible. We have been in the hospital for 11 weeks now and it has taken quite a physical and emotional toll on us. Every day, we deeply absorb the unbelievable generous support we have received from so many and that by far has been what has allowed us to survive this terrible process relatively intact.
Bear with me as this entry is quite long but I wanted to try to explain at least some of the journey we have been on the past several months (really, the past several years).
Under the right conditions, a lot of hard work and a little bit of luck can go a long way. For 8 years, this premise worked impressively well for our family (as it does for many families who are born in the right places at the right times). We were humming along, leading hectic but happy lives. And then the luck ran out. Our 8 year-old daughter drew a very short straw, which began a very long medical quest. With a lot of hard work we would find the answer. But we failed to fully consider the luck part. When one initially gets thrown into the medical system, especially when the diagnosis is elusive, there is an unbelievable amount of guesswork and luck of the draw at play. Our first few encounters with medical specialists were deeply discouraging. One was so pathetic it was comical. It’s as if we were drawing nothing but nonwinning numbers in the medical specialist lottery.
Fortunately, we always had one doctor who kept us moving forward – first our pediatrician, then our integrative medicine doctor, and finally our immunologist. Doctors invested in Kyra’s care, helping us maintain hope along the way. But Kyra’s case is so complex that it requires multiple doctors who can work through the diagnostic process and bring her back to health. Prior to Kyra’s hospitalization, we were trying, in a piecemeal fashion, to build a team of doctors but this is very challenging to do on an outpatient basis. For one, highly competent doctors are busy doctors so getting on their schedule often takes time, even when one’s primary doctor is helping to move the process along. Second, solving (or at least better understanding) a case like Kyra’s demands a lot from doctors. Doctors, being people, respond to this challenge in a variety of ways, not all of them rational or helpful (I refer you to the article written by Laura Hillenbrand that I posted a while back for a sampling of nonsensical medical thinking). We needed doctors who were up for the challenge.
There also are many elements of luck with regard to what types of disease processes receive proper research and medical attention at any given time. Many things have to fall into place for rare diseases to be better understood – researchers need to receive funding to study the disease process, technologies have to be developed to assess and diagnose, and treatments need to be developed. All of these things, and more (including having at least one influential person who can help raise funds and draw attention to the disease), need to occur before breakthroughs are possible. And breakthroughs are rare. Usually the diagnostic and treatment process is more like a slow slog, with many dead ends along the way. Because Kyra’s condition is rare, we are at the mercy of the current state of knowledge of the medical and research systems. We are just hoping that we get lucky and some group of researchers somewhere makes headway on the type of immunological dysfunction that Kyra has fallen prey to. Or, one of the many doctors we have interacted with will go to a conference, or talk with a colleague, and will learn of other individuals who have a similar story to Kyra’s. Kyra’s case may be rare, but across a population (a city, a country, a continent), there will be others like her. We just need to find them. (One of the children who frequently has to be admitted to P/SL has a condition that has afflicted only 39 people worldwide.)
Tim and I have to keep pressing forward because one never knows when luck will change. As luck would have it, we landed at Presbyterian/St. Luke’s 11 weeks ago and just happened to land a fabulous admitting hospitalist. We then were introduced to more fabulous hospitalists (and specialists) and, together, they have worked very hard to provide the best care possible for Kyra. (In April, we went to Children’s for 10 days for very specific diagnostic purposes but once those were complete, we opted to return to P/SL for the remainder of Kyra’s hospitalization. Having full confidence and trust in a team of medical providers is crucial, and after a two and a half year journey, we finally have that piece in place at P/SL.) So we finally have a team of doctors who are all working on drawing a longer straw for Kyra. Like children who play games of chance, we are going to keep playing until we get a straw that let’s her win the game (with a very flexible definition of “win”).
Kyra still does not have a specific diagnosis that explains the chronic nature of her illnesses. However, headway has been on made on several fronts. Below I have described what we currently know about her medical condition(s).
The medical status
As Beth mentioned in her latest update, our GI doctor found two pancreatic stones when he performed an ERCP (Endoscopic Retrograde Cholangiopancreatography) last Tuesday. Fortunately, it just so happens that the ERCP, which he was performing for another purpose, is the procedure they use to flush stones so he was able to clear them that day (I was going to make a “one bird, two stones” joke and then realized it doesn’t really work but I’m keeping the phrase in anyway). Kyra’s still has pancreatitis but her lipase numbers definitely are trending downward. She also is receiving some relief from the pain medication, which was not happening at all earlier. Chances are, the stones were creating a level of pain that no known pain reliever could touch. She still is throwing up every day and feels ridiculously nauseous but these symptoms should diminish as the pancreatitis gets better. Kyra is receiving feeds through an NJ (Nasojejunal) tube and if that goes well, they will transition to an NG (nasogastric) tube and then we hopefully can reintroduce food the old fashioned way. Kyra has not had anything to eat since February 10th, when she began 10 days of violent vomiting. She actually lost her appetite back in November, which is when low grade pancreatitis probably developed (loss of appetite is a classic symptom of pancreatitis). Things look very promising on the GI front. There is nothing to suggest that Kyra will not make a full recovery from a GI standpoint. The only issue that may arise is chronic pancreatitis (which is no small issue) but several hospitals are making remarkable headway in treating this disease.
In early April, some test results assessing Kyra’s complement system came back remarkable (unusual). Her classical and alternative pathways showed deficiencies and she also showed evidence of circulating immune complexes. The tricky part is that immune complexes can build up in the blood for all sorts of reasons. They can be caused by viruses, medications, IVIG treatments, autoimmune diseases, etc., so the immunology doctors are not quite sure what to make of them yet. Immune complexes often are indicative of autoimmune diseases but Kyra does not show the markers for any of the well-known autoimmune disorders (lupus, rheumatoid arthritis, etc). I believe there are over 80 known autoimmune diseases so it may be that she has one of these (and the markers just haven’t shown up yet) or she has an autoimmune disease process that has yet to be formally recognized. Kyra’s symptom picture (unbelievable achiness, headache, temperature dysregulation, malaise, and cognitive impairment, cyclical patterning) is very consistent with an autoimmune presentation. The immunologists want to retest Kyra when she is feeling a bit better to determine whether the same pattern of test results emerges under different conditions. We are continuing to gather as much information as we can on complement deficiencies and various complement researchers have been consulted about Kyra’s case. We believe that this piece of the puzzle may ultimately lead to a more thorough explanation of Kyra’s illness but we have to wait and see.
The thank you
We are fortunate to have good health insurance but as anyone who knows anyone with prolonged and/or serious medical conditions knows, the costs associated with premiums, deductibles, and copays are rising. Moreover, hospitalization costs are not fully covered, for reasons that no one can fully explain. One look at an Explanation of Benefits form illustrates how convoluted the medical billing system has become. We also have to plan for Kyra’s future care and all that may entail. When this fundraiser began, we thought that the contributions would reach a few thousand dollars and for that we would have been so grateful. We have been absolutely stunned (and brought to tears) every time we visit the website or receive an update about funds that have been raised. You have rescued our family when we needed rescuing the most. We wanted to let you know where those funds have gone and where they will go in the future.
· You have paid for our food and lodging since being at the hospital. Tess is not allowed to stay overnight at the hospital so we hold family slumber parties at a nearby hotel when she comes down to visit.
· You allowed us to pay off all debt that had accrued because of Kyra’s condition.
· You are allowing us to pay the medical bills associated with her current hospitalization.
· You are allowing us to create a savings fund to pay for future costs associated with her condition (examples include tutoring that won’t be covered through the school – Kyra missed all but one week of 5th grade; Physical Therapy; Occupational Therapy; Acupuncture; etc.)
· You are allowing Tim and I to continue to work by helping us pay for childcare costs. We want to continue working as I love my job (Hi Lew ) and Tim loves his job (Hi Andy and Jason ) and we need to continue working so that we have two layers of insurance protection for Kyra. We are hopeful that Kyra may qualify for some Medicaid waivers as well but there are waitlists for those. There are so many intangibles associated with your generosity as well. Because of your financial and emotional support, we are as sane as we can be given our circumstances. Most importantly, Grandma Lois, Tiana, and Tess do not have to panhandle on one of our neighborhood street corners or run a perpetual lemonade stand (which of course would really be a front for a more seedy, but more lucrative, business. Grandma Lois would just have to modify her brownie recipe). So, you have kept our youngest, and her caretakers, on the right side of the law as well