The Sasha Sanction

At the start of an Alagille Syndrome care plan, considering the risks of surgery where more than one organ is defective, especially where one organ is seriously compromised as with pulmonary atresia, the family meeting should include cardiologist, gasterointerologist and palliative care service to establish parental consent for the surgical plan.
The word sanction suggests authorization and consent and true consent is best achieved after consideration of all viewpoints. Understandably interdisciplinary family meetings are logistically hard to setup. As Sasha did not have an initial family meeting of the two disciplines and palliative care was introduced not at the outset and not before the second palliative surgery but when she was dying, the Sasha Sanction suggests children with complex care needs and all Alagille Syndrome children with serious liver or heart decease would benefit from an interprofessional family meeting at the start of care and before each surgery. The Sasha Sanction is a credo for some complex care plans and should be an axiom for children with Alagille Syndrome and pulmonary atresia by presenting parents with the prospects of a childhood with multiple successful surgical interventions alongside the risk of both sudden death and the least palatable outcome of a lingering death due to surgical complications.

Study finds poor outcomes when pulmonary atresia presents in Alagille Syndrome

An Australian study published almost a year after Sasha died reviewed 5 cases of Alagille Syndrome and pulmonary atresia, Sasha's combination of defects, over two decades. The study confirms that surgical intervention can extend the child's life but that most children will die in their second to fifth year. I believe we honor these children and help the families when professionals clarify that these children are palliative and also clarify that death can be sudden or lingering over many months.

Pulmonary atresia (PA) is a rare presentation in AGS, but we were impressed by the poor outcome of such infants following review of our institutional experience over the last 20 years.... Five cases of AGS with PA were identified (see Table 1): 4 with PA and ventricular septal defect (VSD), 1 with PA and intact ventricular septum. Four of 5 patients (80%) have died as a result of cardiac disease, and the remaining individual is receiving palliative care....It is not clear why the patients with AGS who received systemic-to-pulmonary shunts did not show evidence of pulmonary arterial growth. In the 3 who had generous collateral flow, initial palliation was deferred, but in the current era we would aim to augment pulmonary blood flow as soon as possible to achieve what pulmonary arterial growth is possible. Unfortunately, even when this approach was aggressively employed (case 1), satisfactory pulmonary vascular development has not been achieved.

The negative impact of Alagille syndrome on survival of infants with pulmonary atresia J Thorac Cardiovasc Surg. 2007 Apr

The Sasha Sanction for fragile, multi-organ impacted Alagille children

1. In the early workup of the Alagille care plan to accomodate liver and heart defects, where transplant is not supported and heart defect is serious, the family meeting should include cardiologist, liver specialist and palliative care to establish parental consent for a surgical plan.
2. Before each surgery, this family -staff group reviews the history of adverse events during catheterizations, prior surgery and any other proceedures to consider the degree of cardiac intervention and surgical stress the child can tolerate.
3. The Sasha Sanction gives parents momentary permission to reconsider their own and the institutions impulse to surgery; it serves as the flight safety movie where you learn of the exits in case of crash; it puts up the flag, at this precise moment, that surgery can lead to life, death and lingering death.
4. The Sasha Sanction recognizes as particularly fragile and honors Alagille children with the serious heart defect pulmonary atresia and liver defect of unknown magnitude.

Background
Since parents are uniquely vulnerable to hope for continued life of a child that will otherwise die, it is not sufficient that a cardiologist secure a cardiac surgeon's agreement to undertake surgery. Parental consent to and authorization of surgery is best achieved after reviewing: risk and benefit to both organs, extent and duration of surgery, health of liver and all adverse events such as internal bleeding and surgical reentry, bleeding at the site of a catheterization, blood pressure drop during exturbation and any single incident of haematemesis (vomiting blood) suggestive of varices or portal hypertension. The final review of adverse events improves patient safety and empowers parents. Small incidents can suggest patterns in combination. The very process of acknowledging adverse events in complex and lengthy care removes the veil that undergirds the power imbalance between parent and staff and expands parents capacity and partnership with staff over feelings of diminished capacity, vigilance and distrust when incidents are not debriefed or, even worse, not acknowledged as important.

Interprofessionalism essential for family centred care
We like to think Sasha's cardiologist 'got us'. She carefully explained options and risks, considered our questions and viewpoints and even offered us a forgiving ear for our needed attempts at humor. Nonetheless, it is mission impossible to ask one physician to navigate this storm alone, each time, without adding occurrences of deep parent regret and doubt about one of the most devastating losses they will ever experience. In the quiet of the clinic we made our decisions, and later I wondered if we had asked enough questions. It is now so clear that these questions should be raised by the institution with a unique amount of experience, not parents for whom this is a unique event, and the institutional vehicle at Sickkids for consideration of the most complex decisions is the interprofessional family meeting. Hearing the separate voices of other disciplines values these disciplines and may encourage deeper consideration at a time when fatigue and grief can impair decision making. The simple absence of other services can create the impression that there is no issue to consider or even that one service is less confident in the capacity, speed or focus of another service. In Sasha's memory, I asked her primary physician to introduce palliative care in similar cases at the outset or before surgery and in her practice I understand she has made this happen, allowing parents, in her words, to consider when palliative care may be "a good option, not just the last option".

Good death
The palliative service offers supports when a child is expected to die within six months and is a consult service. Any parent can request a palliative consult however they may also feel that this would be 'giving up'. Only more routine, earlier introduction of palliative care can assuage that guilt. However there are very few areas of the hospital that refer palliative care in the absence of parent request due to severity of conditions. Palliative service staff can explain to parents that modern medicine views the relationship between recovery and dying as a wiggly line of ups and downs and not a straight diagonal where more palliation equals less life. Children can live, die suddenly, serially crash and be resuscitated or, most distressingly, suffer complications resulting in a lingering death. Staff can explain that parent hopes and dreams of recovery could lead to consideration of interventions that may keep the child alive in greatly diminished capacity. Staff can confirm that families understand they can bring their child home to the place they most love, that it can be done well and that it can give the child and family a very special final time together. Staff can share that Sasha rebounded in spirit immediately upon her return, decided to live another 5 weeks and that her parents wished they had brought Sasha home earlier.

Dedication
The Sasha Sanction is dedicated to Sasha's primary physician who was available at every step of Sasha's care and who redoubled her efforts, tirelessly rounding up multiple services, when Sasha got sicker. Sasha and her family were privileged to have a cardiologist as competent, empathetic and well positioned to effect action.

Pillow Angel: freezing growth

When I first read about Ashley I could only respect the incredibly difficult situation she and her parents were in. Aside from the sympathetic overviews of the ethics and critiques of the parents choices and the incredible respect for the care they put into explaining about Ashley on their blog I thought of a fragment of a conversation we had during one of Sasha's clinic visits as we waited in her cardiologist's office: if only she didn't grow anymore she wouldnt need more cardiac surgeries.