Choosing A Special Needs Child, Ctd

A reader writes:

I’m hydrocephalic, and the condition was able to be treated at birth through a shunt. (I was born in 1976). Let your reader know that the condition is fairly “fixable” now in certain instances, if detected early. My mother spent many years in fear that a specific painkiller she took during pregnancy contributed to my condition, but it really appears to have been a one-off in our case; my three siblings are unaffected. I have to say that I was unaware that hydrocephalus could be carried genetically in this manner, but it makes sense. However, I don’t know what my parents would have done had modern technology been available, or indeed what I myself would have done in this instance. It’s a personal decision, and I imagine the severity of what the doctor detects will dictate what counsel is given to the parents.

Another addresses Andrew Soloman’s piece:

I am a pediatric intensive care physician who took care of a child with trisomy 18 who ultimately died, and I want to share my perspective on the issue of caring for a child with severe genetic defects. Trisomy 18 is quite different from trisomy 21 (Down Syndrome) and, in general, has a very grim prognosis. With this chromosomal abnormality, multiple organ systems can be involved (neurologic, cardiac and digestive tract commonly) and until relatively recently most institutions did not recommend intervention. Moreover, in children in whom interventions are performed, most will still die before reaching one year of life.

The child who I helped take care of had a malformed brain with hydrocephalus and also had seizures.

The suck-and-swallow mechanism was not intact so the child had to be tube-fed. There was also a heart defect that required surgery with cardiopulmonary bypass. The child had a malformed airway and had to have a tracheostomy placed to undergo the surgery. The digestive tract was malformed, the stomach was so small that it couldn’t hold very large volumes and therefore the child had to be fed small amounts continuously for 24 hours per day.

The mother for this child received good prenatal care. She was counseled about the trisomy 18 diagnosis and opted to carry the child to term. Once the child was born, she was a strong advocate for her child and wanted every medical intervention performed that could potentially prolong life. The child spent its entire life, 3 months, in an ICU.

After the heart surgery and tracheostomy placement, the child couldn’t be separated from a mechanical ventilator and was dependent on a breathing machine. There were complications after the heart surgery that required placement of a pacemaker. In adults, pacemakers are hardly noticeable. In a 5-lb child, the device takes up half the abdomen. This became a serious problem. As a result of her small stomach and inability to tolerate adequate caloric intake, she was malnourished, had inadequate fat stores and the device began to erode through her skin.

At times we supplemented her with IV nutrition with central venous lines. These IVs that go into large vessels have significant risk associated with them, including infection and serious blood clots. As a result we don’t leave them into too long. This meant were constantly sticking this child with needles trying to get IVs in superficial veins.  In addition, we also had to draw blood for labs to monitor responses to therapies. IV placement and phlebotomy can be challenging in otherwise normal children much less one who is this ill.

I have no doubt this child suffered. A lot. We provided drugs to help alleviate pain and anxiety. This child became dependent on opiates and benzodiazepines. In light of all the other medical problems, this was relatively small one. However, if the child wasn’t sedated, the child was miserable.  And if we tried to reduce the medications, the child had symptoms of withdrawal.

The mother never left the bedside. She was clearly bonded with her child and the child was definitely soothed when the mother was there. Because the child was frequently unstable, the mother did not get to hold the child very often. The mother learned how to take care of the tracheostomy, the feeding tube. You couldn’t ask for a more attentive parent.

There were costs of her devotion, however. The family lived some distance from the hospital and this mother had two other toddler-aged healthy children who she didn’t see very much because she was always in the hospital. While she and her husband agreed to carry the child to term, they were on very different pages regarding which medical interventions to pursue and for how long. There were financial hardships including getting to and from the hospital, childcare, etc. The child ultimately ended up acquiring a serious pneumonia and cardiovascular instability and sepsis. The mother decided to compassionately stop medical interventions and we disconnected the child from the ventilator and the mother held her dying child.

In this case, the mother chose to take this on. Even in light of all the bad news we physicians bombarded her with, she pressed on.  She joined an online support group of mothers of children with trisomy 18 children. She told us many times that we, the medical team, couldn’t take her hope away. I agree with her. I didn’t want to take her hope away. That isn’t my role nor do I want it to be. I can’t provide false hope either. The struggle I had, and continue to have, is did I do the best I could in treating and advocating for this patient? This child spent its entire life in an ICU, drugged, unable to be held for much of the time, hooked to machinery, being poked and prodded with needles.

From a medical decision-making standpoint, we do not operate in a patriarchal system. The presumption is that parents, unusual circumstances not withstanding, will make decisions in the best interest of their child. Children, almost universally, cannot have advanced directives, and with babies, the point is moot. I have no doubt that the mother believed she was acting in the best interest of her child and I also believe the medical team addressed suffering and quality of life with this mother.  While medical futility applies in a very small number of cases, we wrestle with the moral and ethics of medical interventions all of the time.

The legislation being proposed in North Dakota could have very real costs, in every sense. I’ve provided only one example; there are many more like this. Not everyone will have the resources that the mother I describe above, as limited as her resources were. Divorces are very common in families who have children with serious medical conditions. There is also a very serious economic toll both to the individual family and the healthcare system at large.

Of course all of that pales in comparison to the emotional and physical pain and suffering that a patient and family go through when caring for a baby with a devastating and terminal diagnosis. It is one thing for families (or a mother) to chose to take this on; it’s quite another to force them.