Early in twentieth century the Balkan Peninsula was subdivided into a number of countries; and so was born the term Balkanized. The term has come to be used when things are piecemealed into small and often competing units.

I think some day when the genome gets read for the price of good meal, your doctor  may say I don’t have the time to talk to you about all your missense, nonsense, alterations, inversions, deletions and copy variants. As one scientist said we will soon have cheap scan with a million dollar consult. But you will say that I have an autism spectrum disorder (fill in a blank) and the doc will say: “yes, but the last 25 patients walking out of here had that label too, but all had a different variant and we have to treat each one differently”. We may be on our way to being Balkanized, in a diagnostic way, if you will.

This was a week of frustrating research and writers block. But something beautiful did happen, Haley played hooky from school and I got to have her for the whole day. She fell asleep about mid afternoon before mom came to get her and I stole some time holding her while she napped. I kept staring at this girl and wishing I could see a cure for CDK5 mutation. I realized that this was the next part of the journey and a frustrating one, not only for me, but all of us. Haley was four at the time, relatively healthy, strong and thriving, at least, since June when we got control of the tonic clonic seizures. During this time I cruised the web looking at research, even took in lecture at the College of Nanoscale Science and Engineering of the University at Albany. It was a fascinating lecture on bio-engineering. I did it because I hate being helpless. I hold this beautiful child and wish I had a fortune to give away to fund a cure. But reality gets in the way, that’s not the way it happens. If that were so, Bill and Melinda Gates would have bought a cure for something. As it is, they seem to be trying to put their money to places where it makes differences now.

What you find when you really dig into the research, is that an enormous amount of exciting things are happening, but the complexity is daunting. It is happening because of the enormous impact of neurodegenerative disorders The University of Pittsburgh, Neurology Department puts it this way:

It is estimated that approximately one in four Americans will suffer from a neurodegenerative disease, and virtually all Americans will have a family member with one of these conditions. Unfortunately, the underlying mechanisms of neurodegeneration—and how they lead to disease—are not well understood. The complexity of these diseases makes it impossible for any single scientist to find the cause or cure. Instead, it will require an integrated, collaborative, interdisciplinary approach—involving interactive groups of scientists and clinicians. http://www.neurology.upmc.edu/pind/

Those of us who deal with these beautiful children, learn that they are ready willing and able to move ahead in their lives if we can just remove the roadblocks. We also know that they are our equal in their zest for life. And that zest we share is a most beautiful gift.

Ed Fennel

Grandfather to Two Rare Disorder Granddaughters

“The test of our progress is not whether we add more to the abundance of those who have much; it is whether we provide enough for those who have little.” — Franklin D. Roosevelt

No lesson have I learned so clearly as this one. However, it was not learned from FDR, it was learned from my granddaughter, a child of 8 with a disorder that has stolen her speech, her mobility, her ability to care for herself. Yet she refuses to let it steal her mind. If you are patient and lean close you can hear her. We know for certain she hears us. And for certain her teachers hear her. Why just the other day she told (computer assisted) school staff that the braces used for standing hurt her feet. Lean in close to your brothers and sisters like Haley – they are there, just like everyone else, needing the same thing we all need: recognition that we are here and share the same needs.

Ed Fennell
Poppy to Two Rare Disorder Children

AHCJ | Resources: Reporting on how, why hospital superusers account for bulk of health-care spending.

A recent Children’ Hospital Association study reported that 2 of 3 children who were medically complex were enrolled in Medicaid. A recent article in Pediatrics reported that in North Carolina medically complex children, although numbering only 5% of enrollment represented over 50% of the cost for the pediatric Medicaid population. The standards utilized by the Children’s Hospital Association to define the medically complex child were based on CRG groups 5b-9. The report noted that the this group of patients  receive the majority of their care at a tertiary children’s hospitals.

As a caregiver of two complex children and an activist who interacts with numerous others who give care to the medically complex those observations reflect our universal reality. That being the case I would hope that somehow we could influence the management of  these tertiary care centers to provide practice models that are suitable to children with complex medical needs.

The Heart of a Saint – YouTube.

40 statistics on physician compensation.

It is gratifying that the first stated Principal is Care Coordination. My focus is the care of children with rare and/or medically complex disorders. In reviewing the draft and its principles I do not see any reference to the pediatric population that is in search of its diagnosis for their rare disorders. There are estimated to be 30 mil Americans afflicted by rare disorders, the majority children, who search for a diagnosis for years (7.5yrs-recent Shire Drug Report). These diseases are complex in themselves since 80% are genetic and the majority lack cures and therapies.

When they are finally diagnosed they join the ranks of the long term chronic and complex population, often for life. A recent Children’s Hospital Association Study asserts that 6% of the children on Medicaid represent 40% of the cost. The 6% represent the population in CRG 5b-9 (Clinical Risk Groups). The definitions for CRG 5b-9 reveals that fulfilling the State’s vision of family primacy in care coordination will require some major cultural changes in the continuum of community care. To serve this population will require an acknowledgment that the network MUST incorporate the State’s top children’s hospitals. It is not surprising that clinicians, case managers, and other supportive services that attempt to support families and children in their home, find relationships with these large complex institutions daunting at best. They are, more often than not excellent at trauma and acute care; however prior to admission they seldom have been part of the continuum of care and therefore in many ways ill prepared to join the proposed High Fidelity Wraparound Care Team. It would be rare to have the ambulatory outpatient team integrated into the inpatient team. The isolation of inpatient creates an environment that severely impedes the care continuum at three levels: one, the admission lacks the critical input of the outpatient team; two the child and family witness the brunt of this neglect and feel isolated from the child’s care, as they are customarily only passive witnesses at bedside; and third, the discharge process imposes further trauma, when the enormous assets of the team present at the bedside, evaporates. It is as if the most critical link in the continuum of care is available for only one time single events, after which lines are cut. The very definitions of CRG 5b-9 are testimony to the seriousness of this gap.

1. Chronic & Complex (CRG Statuses 5b and 6) – Child with complex conditions that requires pediatric sub-specialty multidisciplinary care for greater than one year. These patients receive the majority of their care at a tertiary children’s hospital and frequently require diagnostic and therapeutic medical and/or surgical procedures. These patients require a guided transition to the adult health care world for surveillance and management of complications.

1. Critical (CRG Statuses 7, 8, and 9) – Child requiring intensive, multidisciplinary care for >3 years, that enters the health system with a life-threatening or progressive condition. These patients require ICU care and ongoing procedural and sub-specialty care at a tertiary children’s hospital. These children require a guided transition to the adult health care world due to ongoing, lifelong medical conditions.

Source: Children’s Hospital Association

Parents, such as me rely on these hospitals to stabilize our children at their baseline level and treat them when they diverge from it. We struggle along with our outpatient support structure to maintain that baseline. We need an infrastructure that marries the community support structure with the care teams of the large hospitals. Their current relationship is thin at best and antagonistic at worse.

This NYS Health Home Initiative for the Pediatric population has a truly unique opportunity. By recognizing that this small population as a major driver of cost and a population that is extremely diverse it can widen the boundaries of care coordination to include all pediatric care assets in the State. Consider that in any region the local children’s hospital own the VAST majority of assets to serve the CRG 5b-9 population. The PICU and NICU beds are not at the pediatricians office or community clinic, nor are the pediatric sub-specialists likely to work anyplace other than the children’s hospital.

Reforming Medicaid for medically complex children was addressed in an April 2013 article in Pediatrics. It could provide a guide for creating a network of our university based pediatric hospitals. This initiative could be basis of a partnership to address some of the problems these hospitals face:

Children’s hospitals play a central role in our child health care system. These hospitals face unique challenges under health care reform. They care for children with the most complex medical problems but often are not reimbursed for good preventive care, care coordination, or quality.

This partnership could also utilize NYS’s ACO legislation to link these hospitals in service to this population and thereby create a national model.

 

It is estimated that there are 3 million children with complex medical conditions; two million of them on Medicaid. Those children represent only 6% of the children on Medicaid, yet represent 40% of the expenditures. The literature indicates little is known about this population, despite their large impact.

From a national policy point of view, the neglect of this population has a lot to do with states’ rights, each state playing its own pickup game with children’s health policy. Children’s needs become victims of electoral politics driven by party affiliation, divergent ideologies, nationality, race, ethnicity and religion. Personally I wouldn’t be surprised if our national political inertia lasted at least another decade; but it is my belief that states of great diversity, size, and rich in medical assets could lead the way to setting the standards that one day will be national.

As these states venture forward they will prevail if they tackle the fundamental systemic problems of serving this population. One of the greatest systemic problems in medicine today is its proclivity to drift towards what one of my MD colleagues’ calls single event medicine.  I just had a hip replacement which I score a PERFECT TEN.  However, I care for two granddaughters with complex medical needs: one has a neuro-developmental disorder, of which there are 100 cases reported in the literature, and it is accompanied by intractable epilepsy; the second is short bowel, having lost over 95% of her small bowel and 75% of her large bowel at two days old. Their care takes a multi-state medical team, comfortable with complications not seen before; and comfortable with each other. As near as patients, caregivers and parents can tell medicine is not a team sport especially when it comes to such rare diseases and medically complex cases.

Yet, I look at regional children’s hospitals and I see the potential for networks that can raise the bar for these cases. But I can tell you as grandparent who cares for two children with rare and complex needs we are fed up with single event specialists, especially those who lack talent in the areas of collaboration and communication. We know the cures may not be available that therapeutics and medical interventions may be limited. We see the frustration on the faces of our providers who feel inept because they don’t have all the skills. We also see their frustration when there may be no one to pass the case to and when they do the ball is often dropped. We all sense that it is not just us who are frustrated; it is the practioners as well. And that is the systemic problem for children with complex medical needs representing 40% of the 0-18 Medicaid expenses.

Our need to balance our health care budgets now, does not rest in future solutions; its rests on current team work. To begin this process we need recognize that our regional children’s hospitals are the hub. In my home state of New York the regional children’s hospitals control the overwhelming majority of assets necessary to handle these complex cases. The heart of any initiative begins through the collaborative efforts of these rich and valuable resources. The first state to do this will do a valuable service for the nation’s children.

Why Not New York? The timing is perfect. They are currently initiating a Medicaid Health Home for Children and it could be a national leader.

 

Recommended Reading

Reforming Medicaid for Medically Complex Children

Extrapolated Summary of Medically Complex Children and Total Children in Medicaid Annual Utilization

Annual Utilization per 1,000 Patients, age 0-18 by Health Status Group and Site of Care, Medicaid