Do you know what the plural of paradox is? I had to look it up, and sadly it’s just boring old paradoxes. I was really hoping for paradoci or paradocorum or something else a little interesting.
Because in health care, especially in the rare disorder world, and even more especially for whole-life/whole-body disorders, there isn’t just one paradox for each patient, but many. I mentioned the weight paradox recently, where the disorder first contributes to excess weight and then is worsened by it, leading to even more excess weight.
Today’s paradox involves the team approach to health care for chronic hypophosphatemia. Team approaches are something of a fad in medical academia right now—a quick search at pubmed generates almost 3,000 articles with “multidisciplinary team” as a key word, compared to about half that for “lived experience”— but even discounting that element, it’s striking that every single one of the standard of care articles about XLH treatment recommends an interdisciplinary team approach. There’s absolutely no controversy at the theoretical level, but it’s a mess at the practical level.
The main challenge involves finding someone to coordinate the team. Naming the various specialties that should be involved (e.g., orthopedics, pain management, occupational/physical therapy, talk therapy, dental) is useful information, but the specialists don’t generally just show up uninvited in the main health care provider’s exam room to add their two cents. There are a few centers of excellence where a wide range of specialists with rare bone disorder experience can be seen in a single day (or at who at least talk to each other regularly), but the multi-specialty settings that I know of tend to be available only to pediatric patients. And it’s unlikely that we’ll see this kind of institutional team treatment become available for the majority of XLH patients (even if you expand it to “rare bone disorder patients” instead of just phosphate-wasting disorders), given that a significant percentage of XLH patients can’t access even ONE specialist with the relevant experience, let alone a whole bunch of them!
We’re left with the reality that, in the absence of access to one-stop-shopping for all your XLH needs, someone is going to have to volunteer to coordinate all the appropriate specialists. There are basically two options: the primary care provider or the patient/caregiver. Neither one is ideal.
The primary care provider (PCP) is generally expected to coordinate care for their patients, and that’s fine for something like heart disease or cancer, where there are clear protocols and referrals. All the PCP needs to do is send the patient to a cardiologist or oncologist, and then make sure the patient follows up with the relevant specialist. I’m sure there are some add-on referrals that are less universal or more dependent on the specifics of the patient’s experience, like pain management or physical therapy, but judging from my cardiac experience, there are still clear policies and procedures for either the PCP or the specialist to make those referrals, and there are standard timeframes and frequency and triggers for the referrals. In other words, there are systems in place, much like the systems to prevent drug interactions, to assist the PCP and/or specialist in making those referrals and following up on them.
There’s no such policy/protocol/system to help a PCP oversee the multidisciplinary team for XLH (or rare disorders generally). And unlike the PCP’s understanding of the teams needed for cardiac/cancer care, there’s nothing similar for rare disorders. In fact, the PCP is unlikely to have even the most general idea of what the team for chronic hypophosphatemia should be composed of or when various specialties should be consulted. A PCP who doesn’t even know the basics of XLH (and who doesn’t have the time to learn them) isn’t going to know that a team approach is needed, and even if they know that much, will they remember the less well-known specialties we may need, like occupational therapy or hearing tests? If they have a cheat sheet for all the possible specialties, will they know when the specialty is needed, since we don’t need all of them during every phase of our lives. It all depends on the patient’s unique circumstances, so it isn’t possible to create a one-size-fits-most system that a PCP can follow without fully understanding the underlying issues.
You might think that one of the specialists in XLH—most likely the endocrinologist—might serve as the person coordinating the other specialists. And in some cases, I’m sure that does happen. It’s just not the norm, and it takes a truly extraordinary endocrinologist, probably one in a setting where there are already teams set up for the relevant disorder, to be willing to take on the work that their training tells them is not their problem.
That means, for the vast majority of patients, the patient (or caregiver) is going to be doing the coordinating. And, once again, doing it without a lot of guidance. Most XLHers (other than the parents of a spontaneous case in the early days of coping with the shock of the diagnosis) know that there are a bunch of relevant specialists, but what we don’t always know is when we need to engage them. We’re likely to have a better idea than the PCP does (I spent years fighting with my PCP to stop her from referring me to a pain specialist, which I knew from experience had nothing to offer me at that stage of my XLH), but we aren’t highly trained in all the specialties we’ll be coordinating, so there can be times when we don’t know that we could benefit from specialized advice. I consider myself fairly advanced in my understanding of the various issues related to XLH, but I just learned something new in a response to the recent weight paradox post. If we’re planning a weight loss program (beyond the standard “eat less/better, move more”), it may be beneficial to discuss it, not just with a weight-loss specialist, but also with our bone-metabolism specialist (endocrinologist). Some options may interact in less-than-ideal ways with our weird metabolism and/or our XLH treatment.
I don’t have good solutions, but there may be room in my planned series for educating PCPs to include a proposed flow chart for engaging specialists, which patients can use too. I would welcome suggestions for when you have most benefited from the various options (other than dental, which in our stupid system isn’t part of the general health care system, and in any event are pretty much the same as for the general public, just more intense/frequent). I’m thinking we need to have a flow chart for: orthopedics, pain management, occupational therapy, physical therapy, talk therapy, chocolate therapy, hearing tests, neurology (Chiari in kids, spinal issues later on), rheumatology (for arthritis/inflammation), nutrition, weight management, genetic testing/counseling, pregnancy/lactation.
Have you been coordinating specialists for yourself and/or a loved one? Am I missing anything above? Any advice for others?
***
Please note that the author is a well-read patient, not a doctor, and is not offering medical or legal advice.
If you’d prefer to read this blog as a newsletter, sent straight to your inbox, please sign up here.