Symptom Management

Preparing for end stages

Facing Death

One issue that all parents of a child with a neuro-degenerative disease must eventually face is the death of their child. While scientific information and medical experts may describe the typical course of these diseases, each individual case is different, and it is often difficult to accurately predict when or how a child will succumb to his or her illness. Several families have faced situations where health care professionals have predicted a child's death as imminent many times (and sometimes many years) before the actual death occurs.

One way to cope with this uncertainty is to make as many arrangements as you feel comfortable with ahead of time. Decisions such as burial place, type of memorial service and the people you want to be involved can be made in advance and will save you the added burden of taking care of these arrangements at the time of your child's death. Many funeral homes are receptive to making advance arrangements and can assist you in this effort.

“I made no funeral arrangements prior to his death. However, I wish I had. I let others pick out the place for him to be waked, and the people who took care of his arrangements were terrible. I guess I would tell others that, as difficult as it is to face, you should try to make some arrangements or choices earlier on.”

Intervention: Deciding When And How

At some time in your child's life you may have to confront the issue of the “Do Not Resuscitate” (DNR) order. Doctors and hospitals are wary of not doing everything possible to save your child's life (including performing tracheotomies and utilizing ventilators or respirators) unless you have expressly stated that you do not wish these interventions to be used.

It is extremely difficult to make the decision to use “no heroic efforts” to save your child. Even the decision to do everything possible can be a troubling one. In either case, it helps to have the understanding and support of the health care professionals involved with your child. And in all cases, it helps to remember that these are very personal and individual decisions. There are no right or wrong answers, only your answers, and indeed your answers may change over time. You are free to change your mind and alter your decisions.

“We have always known that we would do everything possible to keep Tammy alive, never giving up on the hope that research will result in a treatment becoming available.”

“We never discussed this [DNR] until one day when he was about 21/2, and had to be admitted to the hospital. He was very weak. He was having trouble eating and drinking. We knew at this point it was not possible for him to have any quality of life, and it was at this point we made that decision. It was very difficult to make, and prior to his getting this sick I would not have been able to make that decision.”

“I have discussed the DNR with both her pediatrician and neurologist. I don't know how we'll feel when actually faced with sticking with this decision. Our feeling in making this decision is that it is selfish to keep her alive by extraordinary means. It is not life to be on a machine and unaware. When Rebecca's time comes I pray to have the strength to let her go. For what it's worth, her pediatrician is very good at reminding me of these type of decisions and helping me to stick with it.”

When The Time Comes

Parents are often curious as to just how a child does eventually die. While textbooks on these diseases state that respiratory infections of some kind are usually responsible, many parents report that at some point, “my child's body just seemed to stop working. He stopped breathing and didn't start again.” Interestingly, often a cessation of breathing (apnea) has occurred previously (sometimes numerous times) but there's “just something different” about the last time. Perhaps the difference is that the parent is more ready and less willing to intervene at that point to prolong the child's life. Mercifully, most such deaths are as peaceful as we would wish for our children, and for ourselves.

“The last two weeks of Daniel's life were quite different from what we were accustomed to during any other time in his life. Daniel's digestion completely slowed down to the point where he could not digest his formula or even water for that matter. Danny's temperature was below normal, approximately 96 degrees, also something we had not seen before. It was during this period that he stopped breathing again, but for the first time we did not intervene. Daniel died at 3 a.m. exactly two months before his fifth birthday.”

Guilt

When a child is born or diagnosed with a devastating disease, it is natural to want to “look for someone to blame.” Often, it seems, parents end up either blaming themselves in some way or feeling bad about something they did or did not do for their child.

“As his mother, I felt so bad I had put Tommy through so many tests. Sometimes I still torture myself with my thoughts. I've stopped doing that now. But if I could go back, I would have stopped all unnecessary tests.”

“I felt guilt when I was told that one in four were the odds for my children being born with Niemann-Pick Disease. Gregory was my fourth pregnancy, and my first pregnancy resulted in an abortion.”

“Guilt was experienced with hindsight that we failed to fully enjoy her more outgoing abilities while she still had them.”

“Only rarely did I lose patience and that is what I regret, that I did at all.”

“The guilt I have at times is not exercising Kathy enough.”

As is apparent, the guilt reaction runs the gamut from things over which we truly have no control to our simply being less than perfect people. Whatever the source, rational or not, guilt can be a heavy burden and destructive force. Many parents have mentioned that reading specific books has been helpful to them as they try to deal with and work through their feelings of guilt. Others have been helped by talking over their feelings with other parents, family members, friends, clergy, or professional therapists.