I cannot imagine anything harder than the suffering I see too many parents go through when their child has a fatal, progressive  neurodegenerative disease.  With the diagnosis they lose their child’s future just as suddenly and unexpectedly as if they were hit by a Mack Truck.  However,  instead of the process being over in any brief period of time, it is often drawn out over many years.

Leukodystrophies, Mitochondrial disorders, Inborn errors of metabolism,  Rett Syndrome, Duchenne Muscular Dystrophy and a host of other heartbreaking diseases are a death sentence, only one that is indefinitely prolonged.   In all these examples the child gradually loses abilities he or she once had.  

Sometimes the disease hits hard early, and then lingers very slowly as the child is trapped forever in infancy.   Some require mechanical ventilation very early in life, which they never overcome and more permanent artificial airway, a tracheostomy, has to be placed.

What is left to do for these children, or their parents?  Because they may have years left, we do all we can to provide comfort.  As they lose the ability to swallow, we place a permanent feeding tube through the skin into the stomach.  Over time, aspirating their own saliva causes more and more frequent pneumonia, until one day, weeks, months, or years down the road, the lungs give out for the last time.

All these measures keep the child from starving from lack of food, water, and oxygen.  They also prolong the agony.  They create a situation where taking away any of those things has the look and feel of murder, even though the means by which these children live is entirely man made and artificial.  To ask any parent to ever pull the plug is beyond cruel.

The care involved for these children is extensive and exhausting.  I have seen it take over parent’s lives.  It is a powerful testament to how deep the parent-child bond really runs to see the lengths some of these parents go.  At some point, there will come a day where this tireless devotion and demand for care is counterproductive and really is prolonging suffering.  From where I sit, at the end of my training, I remain at a loss for any kind of definition of what that point is.

There simply is not any hard and fast rule.  One thing I know, health care workers in general, and physicians  in particularly abhor is the idea of giving futile care.  We hate it, hate it, hate it.  It feels inhumane.  Its cost is extravagant.  From a short term perspective, overworked in the ICU or the ER, it feels like a complete waste of our time.  I have heard resident after resident fume over these patients.  I have even been consulted, not for any neurologic management issues, but to “reason” with the parents about end of life issues.

The problem is, they might decide to make a patient do not  resuscitate, say your goodbyes, shed tears, at set the child in a corner, only to have them limp along living for years.  Without care, the joints get muscle contractures freezing them in place, making movement excruciating.  Hips can even pull themselves out of joint, spontaneously.  Bedsores develop as the skin dies under the patients own weight.  Dystonia may develop, where the neck, arms, or legs are fixed in twisted, painful postures due to brain damage.  If not kept on a regular bowel regimen, constipation may eventually block the gut entirely and cause the stomach to swell painfully.

In these cases, completely giving up on medical care is doing the child no favors.  This is where the pressing need for a DNR is treating distressed caregivers, not the patient.   These parents know their child is dying.  I think we misunderstand when we think they want to put off the hard decisions.  Nobody comes to a place where they are ready to “give up” on a child overnight.  I would be very frightened the children of the world if parents could give up this easily.

Those few that do make the snap decision to cut off care are usually suppressing natural grieving.  They will pay for it later as the grief comes out as anger, abuse, substance use, depression, and self loathing.  More to the point, that decision does not have to be made every hospital stay, for there will be many.  What we end up communicating to these parents is that their child is not welcome here.  We have given up on them.  Please don’t ask us to put ourselves out.

Far too many health care workers fail to understand that palliative care is actually care.  It does not mean sticking the patient in a closet so you can concentrate on those with life worth living.  Instead it is wearing ourselves out, to make what life is left as comfortable as it can be, however long as it may last.

Over time, with support and people to talk too, and physicians to build a relationship with, I have learned parents do progress through grieving to the point where they can accept a DNR.  The greater the support, the sooner peace is made.  The more trust the physician has, the less they feel DNR means giving up on their child, and more they realize it is loving them.  When the guilt is overcome, they are at last freed to savor the long, slow goodbye.

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