all at once All at once The world can overwhelm me There's almost nothin' That you could tell me That could ease my mind Which way will you run When it's always all around you And the feelin' lost and found you again A feelin' that we have no control Around a song some say There's gonna be the new hell some say It's still too early to tell Some say it really ain't no myth at all Keep askin ourselves Are we really Strong enough There's so many things That we got Too proud of We're too proud of I wanna take the preconceived Out from underneath your feet We could shake it off instead We'll plant some seeds We'll watch em' as they grow And with each new beat from your heart The roots grow deeper the branches Will they reach for what Nobody really knows But underneath it all There's this heart All alone What about is gone And it really Won't be so long Sometimes it feels Like a heart Is no place To be singin' From at all There's a world we've never seen There's still hope between the dreams The weight of it all could blow away With a breeze If your waitin on the wind Don't forget to breathe Cause as the darkness Gets deeper We'll be sinkin As we reach for love At least somethin We could hold But i'll reach to you From where time Just can't go...

go on In my rear view I watch you watching the twilight Behind the telephone lines With nothing to prove, or to assume Just thinking that your thoughts are different than mine In my rear view I watch you And I gave you your life, would you give me mine I see you slowly swim away Cause the light is leaving town To a place that I can’t be But there’s no apologies Just go on Just go on There’s still so many things I wanna say to you But go on Just go on We’re bound by blood that’s moving From the moment that we start From the moment that we start I see your perfect little eyes Watch the shadows of the clouds And the surface of the ocean out the window of a plane I get nervous when I fly I’m used to walking with my feet Turbulence is like a sigh that I can’t help but over think What is the purpose of my life If it doesn’t ever do With learning to let it go Live vicariously through You could do the same It’s the least you could do Cause it’s a lonely little chain If you don’t add to it So go on Just go on There’s still so many things I wanna say to you But go on Just go on We’re bound by blood and love From the moment that we start Just go on There’s still so many things I wanna say to you Just go on We’re bound by blood that’s moving From the moment that we start

Jack Johnson, Sleep Through The Static, 2008

Parent involvement in Sickkids NICU remodelling

Today's Toronto Star included the article "Premature Babies Thrive in Cozier ICU" (thanks Janis for passing along) showing parent involvement by Gord and Stacey Archer in Sickkids NICU remodelling plan that will have "bright, noisy rooms replaced by womb-like conditions". The work includes use of sound-absorbent tiles, cushion flooring, task lighting to reduce bright overhead lights, sound monitors at each bed to alert staff if noise levels rise above 30 decibels (they can go as high as 120 db), white boards for pictures, comfortable rocking chairs, painting the room like a nursery; also, staff will be taught "how to spot signs of stress and how to handle the babies with slow and careful motions."

'Stacey and Gord Archer noticed a difference when their son Samuel was placed in a quiet, isolated room after undergoing heart surgery shortly after he was born, seven weeks early, in December 2006. They watched anxiously as their tiny baby grew slowly but steadily over the first several weeks. "Every calorie counted for the little guy," Gord says. "He needed to gain weight, which was only a half-a-pound every 10 days." With his recovery progressing well after a couple of months, Samuel was moved to one of the hospital's nine regular neonatal intensive care rooms, which can house four to six babies each. Located closer to the nursing station and with large windows, his new room was much brighter and noisier. Within days, his parents could see his health begin to deteriorate. "There were a lot of stressors in this room, which prevented Samuel from gaining weight," Stacey recalls. "As a parent, it's so stressful, because you know that every sound is affecting the baby, but there isn't anything you can do." When Samuel stopped gaining weight and developed high blood pressure, his parents asked that he be moved back into the quieter, isolated room. Within days, he began to improve. "He was sleeping better, digesting food better – it was a remarkable difference," Gord says. "It's so much common sense. All the rooms should be like this. They were good not only for Samuel, but for us, too. When the parents are less stressed out, they can connect with the baby better." Based on their experience, the Archers have been giving SickKids advice on how to create neonatal rooms that will help calm both the fragile patients and their frazzled parents.'

Having heard parts of Gord and Stacey and Sam's story from their thoughts and suggestions to the NICU family brainstorm last month, I am happy to see the NICU including them in environmental solutions. It makes sense that reduction of noise and noxious stimulation is very important for preterm children but would it not also help any kids in any ICU environment? While in CCCU I was very sensitive to Sasha's sleep, focusing on environmental noise, however a quick dive into the literature shows it is accepted that critically ill patients have very disrupted sleep patterns, missing certain types of sleep (including REM sleep) as opposed to sleeping little. Non-intuitively, a 2003 study thought environmental noise may account for less than 30% of poor sleep and a 2001 study thought that number might be as low as 10% of arousals from sleep with more disruption and waking up caused by mechanical ventilation, conversations and hands on check of vitals. Generally I found staff made an effort to keep things quiet, as did parents. Once I was irked by a loud TV movie when Sasha was restless but couldn't say anything when I looked over at the dad so happy to have his daughter awake and animated. Once in a CCCU room with only two other children on a quiet weekend morning I was enjoying the soft light and two nurses shared a joke and started laughing - I cannot say they were particularly loud, it simply bothered me and I asked them to please be quiet. Better sleep felt like a sign of recovery. Without doubt, poor sleep effects patients and is a recurring concern and focus for parents. One study suggested giving patients ear plugs.

SickKids Childlife kids and parents music groups are one step closer to reality

Caron Mills of Child Life met with Treasa Levasseur and myself to discuss the how, where and when of a music program. Treasa's experience with kid's music and the music business and her extensive network aligns fantastically well with Caron and my interest in seeing lots more music at SickKids. And the meeting was far more exciting than I could ever envision. Treasa offered to fold in her honorarium into the program budget which will be used for aids, good quality musical instruments and some guest honorariums. She is also open to older kids helping out with younger kids and finding ways to connect older kids with other musicians that they can jam with. While looking at the Reading Room, Caron pointed out that a video can be piped into the rooms. Treasa began to then imagine alternating between video and radio: imagine two IV poles and a sheet with artwork or a puppet show and imagine voice over artists from kids TV shows and kids calling down for requests. We thought it good to allow some time before and after for some chat and start off with 2 groups a month working up to 1 per week in the next few months. After a break over the summer, the program would continue September to summer. Treasa has offered a one year commitment and to also help source kids and adult groups for the atrium stage. Next time you are going into the cafetaria, have a look up and imagine a kids band in silly costumes playing great music on the bare stage.

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.

Frank Gavin on hospital family advisory structures

Frank Gavin, long time member of the SickKids Family Advisory Committee shares his comparative notes on five hospitals with parent advisor infrastructure. Frank offered the NICU Brainstorm to share resources on NICU family advisories or parent involvement and has been a mentor to the parents involved. His practical and theoretical knowledge of family/parent participation is a tremendous asset as SickKids expands parent participation.