Comfort, Caring and Elizabeth
Home Care for a Child with a Neurological Illness
Copyright © 1996 Marla Fine
Revised April 1996
Dedicated to the memory
of Elizabeth Sabine Fine-Gagne
June 20, 1991 - September 29, 1995
“The first time her laughter unfurled its wings in the wind, we knew the world would never be the same.”
You can download a copy of this document in Adobe Acrobat PDF format. Feel free to distribute it to people who you think may find it helpful.
Looking Back-Preface to the 1996 Revision
I began writing this document towards the end of 1993, when my daughter Elizabeth was nearing the age of two and a half. Just after her first birthday, Elizabeth was diagnosed with Tay-Sachs, a fatal neurodegenerative disease. The next year and a half was not only an emotional roller coaster filled with many lows and a surprising number of highs, it was also an intensive course in medicine, home care nursing, team management and public relations. Frustration was something I increasingly felt, particularly when it seemed that we hadn’t been given helpful information soon enough.
That frustration, plus a background in methods and procedures and teaching, led me to write this document in the hope that other children would be able to avoid some of the problems we encountered or, if not, then there would be a variety of practical suggestions for dealing with them. That hope seems to have come to fruition in the two years that we have been distributing Comfort, Caring and Elizabeth . It is a great comfort to know that through me, Elizabeth is able to help many other children and their families.
Elizabeth died at the end of September of 1995, at the age of four years, three months and nine days. I record her age in such detail because I think one of the many things we learned from her was to focus more on each day as it happened and to make the time we did have with her as pleasant and as full as possible. Recently, I heard the survivor of a great tragedy speak of the magic of a boring evening at home and it struck a chord with me. I remembered all those times sitting with Elizabeth on my lap in the sun on the front porch or in our cosy chair in the living room, thinking there was just about nothing better in the world to do. And I know now that I was right, and that we need to value the simplest things, the small hand curled around a finger, the smell of freshly washed hair, a soft cheek, a yawn, a stretch.
I spend a lot of time talking about our life with Elizabeth to other parents in similar situations and to friends and family and other groups. I tell them about the difficulties, about the initial feeling of wanting it all to be over soon changing into deal making and goal setting (just let her make to the holidays or her birthday or a vacation), about wanting to escape especially when I was lacking sleep, about trying to fight something for which there is no cure. But I also tell them about the successes, that Elizabeth lived longer and more fully than anyone expected, that her fourth birthday bash was a terrific celebration, that her passing was fairly peaceful and in a place she loved, that my sons and my husband and I have grown greatly in understanding and compassion through knowing Elizabeth. As a dear friend of mine wrote, “In her short life Elizabeth awakened more love, sympathy, joy and a heartfelt sense of the preciousness of existence than most of us can do in a long one. It is an eviable legacy.”
Our daughter Elizabeth has Tay-Sachs disease. It seems odd how matter-of-factly I can now make that statement - at one time I couldn’t even utter those words without falling into tears. But much has happened since that horrendous June day when Elizabeth, who had just passed her first birthday, was diagnosed as having Tay-Sachs, a fatal neurodegenerative illness. Time, while certainly not capable of healing this wound, nevertheless has allowed us to adjust and ‘catch up’ to our situation. It’s not that the hurt has lessened; it’s just that we have come to focus more on each day as it comes and on making the time we do have with Elizabeth as pleasant as possible. And that is what we hope this list of ideas will do for you - help you to make your child’s life as comfortable and unstressful as possible.
That said, however, I want to warn you that reading through the following pages may well increase your anxiety initially as you wonder about what things you may encounter as time goes on. Bear in mind that each child, each illness and each family’s experience is different. Just like other children, each of our kids has their own make-up and set of circumstances that will shape their life. We’ve had respiratory problems, but then this type of problem runs in my family - in fact our older ‘normal’ son has mild asthma. Another child we know of had difficulty sleeping at night, but so did her siblings. And a third child seemed to have a relatively peaceful time without major respiratory, sleeping or seizure problems. So rather than worrying about what might happen as you read through these pages, I hope you will feel more prepared to cope with your child’s day to day life.
To make this document as useful as possible to as many people as possible, we compiled our list not just from our experiences with Elizabeth, but from our discussions with the other parents in the National Tay-Sachs and Allied Diseases Association of Ontario (our support group), from books and from people in the medical community. We have given some background in each section on our experiences with Elizabeth in order to give a context to the ideas. Please discuss the use of our ideas with the physicians and/or nurses that are helping you care for your child to ensure the appropriateness of the actions. We hope our ideas and experiences will be of benefit to you.
Finally, we are grateful to Dr. Joe Clarke at the Hospital for Sick Children in Toronto for his support and for reviewing this document.
Dealing with Doctors and Other Medical People
Having a good ‘medical support team’ has been crucial to our ability to provide good care for Elizabeth at home. The various people we turn to for support (both medical and emotional) include Elizabeth’s pediatrician, her genetics specialist, their nurses, her physiotherapist, her night nurses, the respiratory technician, the palliative care team and our pharmacist. Some of the things that have helped to keep our ‘team’ running well include:
Elizabeth was breastfed until she was 10 months old, when we switched to bottle-feeding. She started eating ‘solid’ baby food from a spoon at about 4 months. Feeding her solids was often difficult, although we didn’t know why at the time. While Elizabeth did learn to eat semi-solid food from a spoon, to feed herself cookies and to drink from a cup, she was a very picky eater who ate less than we thought she should. I still feel bad when I remember my lack of patience and anger with Elizabeth when she would purse her lips and make the face that meant “That’s it, folks. I’m not letting another bit of food past these lips.” She seems to have had more sense than we did, for at that point she was quite chubby and clearly not lacking calories.
Mealtimes became more pleasant & less tense after Elizabeth’s diagnosis was confirmed and we understood why eating was becoming more difficult for her instead of easier. We found that mushy food was easier for her to swallow than thin, runny foods. (A Fisher Price food mill was really helpful for getting foods to the right texture.) She also came to like finger feeding rather than spoon feeding. Most importantly, we didn’t push the quantity of solid food and we also left time and room for the treats (chocolate cake, cheese buns, peanut butter sandwiches) that Elizabeth really liked.
As time passed and Elizabeth’s ability to eat solids continued to decrease, we began to supplement her milk with small amounts of Ensure with fibre™. She liked the vanilla flavour so much, that she eventually refused plain milk. We gave up on solids completely when Elizabeth was about 17 months old and struggling with the chicken pox. Things that may help include:
Eventually bottle-feeding also grew more difficult. The feeds took longer, Elizabeth began to vomit more and our frustration was increasing. Then, Elizabeth was hospitalized with viral pneumonia. She had great difficulty sucking and feeding, and was put on intravenous feeds. One doctor raised the possibility of a gastrostomy, but it was suggested that we give Elizabeth time to recover and regain her ability to drink from a bottle. Unfortunately, she didn’t have that time. When she came home she was sucking better, but continuing to vomit a lot. After about a week, she was back in hospital with bacterial pneumonia. A barium swallow showed that she was aspirating. We were faced with one of the more stressful decisions we have had to make - switching to gastrostomy tube feeding. While we had largely decided when Elizabeth was diagnosed that she eventually have a G-tube, it was happening sooner than we expected. And even though we felt that it was inevitable and that it would ultimately make her more comfortable and that bottle feeding was no fun at this point, it still seemed to be such a great step backward, such an admission of defeat. And it was. . . . but it was also what was going to keep Elizabeth most comfortable and well and here with us, so we went ahead with the gastrostomy.
In retrospect, the G-tube has allowed us to achieve our main goal: keeping Elizabeth comfortable and at home with us. Because she is well nourished, she is stronger, healthier and able to use her valuable energy for cuddling, exercising and going on outings rather than struggling to eat. It also makes giving medication quite easy. This is not, however, to say that g-tube feeding is the only choice; rather, it was our personal choice given Elizabeth’s condition and our situation. We know of other families who have made different choices (nasal-gastric tube feeding, no tube feeding at all, or a combination) that were right for their children. It is an incredibly difficult decision, but it was helpful to talk to others who had already gone through it. Some of the other things we have done or are doing include:
Constipation and hard, painful stools have always been a problem for Elizabeth. In addition to the discomfort, Elizabeth throws up when constipated. We have tried such things as:
Elizabeth’s weak spot has always been her respiratory system, and she certainly had more problems in this area than many children we know. The main challenge on a daily basis is to help her deal with phlegm, particularly in her upper airways. Her breathing has always been noisy, even as a baby - we used to call it ‘rusty pipes’. She often sounds worse than she is, so I got a stethoscope and listened to her chest daily. In time, I was able to sort through the noises to ensure that there was good air entry throughout her lungs and that she didn’t seem to have pneumonia, which is the thing we worry about the most. So strongly did the doctors stress the dangers of bacterial pneumonia due to aspiration, that we felt that every time Elizabeth spit up or vomited, she would get pneumonia, but fortunately this hasn’t been the case. We also worry that each cold or cough will lead to pneumonia, particularly with Elizabeth’s immobility. Because of these concerns, we have taken a lot of preventative steps and have, by and large, been successful at keeping her comfortable and reasonably well in a quite unreasonable situation. Here are the things we have tried:
Infections (Coughs, Colds, Flu, etc.)
Right from the start, Elizabeth seemed to get every bug that was going around. She had her first ear infection at 2 weeks old, and has kept right on going since then through colds, coughs, croup, more ear infections, pneumonia (viral & bacterial) and even chicken pox. I guess it’s due to a weak immune system, as well as the germs carried by parents who work in a hospital and a school and by her older and younger brothers. Ever vigilant to avoid what we can, we (rather obsessively) do the following things:
For about 6 or 7 months after Elizabeth was diagnosed, we did not have any real problems with seizures. They consisted mostly of what we called “checking out” - rolling her eyes and looking a bit blank for a few seconds. It was when Elizabeth was 19 months old and in the hospital with pneumonia that we started giving her anti-seizure medication . At that point, her seizures lasted about one to two minutes and consisted of facial ticks, limb twitching and irregular breathing. We were told that the seizure drugs would cause drowsiness; however, we noticed that with Elizabeth, they left her more alert overall since they reduced the seizures that made her tired.
From our conversations with doctors & other parents, it seems that the nature of seizures, their control and the effects of anti-convulsant drugs differ from one child to another. The drugs and dosages used will vary. Some parents choose to use very little in the way of anticonvulsants while others move in the opposite direction. Again, it depends on the child, the parents and the doctor.
We have had to increase Elizabeth’s dosages and add drugs as her illness has progressed and seizures became more of a problem. Our main guide with seizure control has been to adjust her medications when the seizures seem to be causing her a lot of discomfort or unease. The medication itself can be a little hard on the stomach so we usually give it to her after she has had about 50-75 mL of her feeding. Doing it this way also seems to slow down the absorption rate a little. It also seems to us that seizures sometimes increase temporarily due to stress (illness, constipation, lack of sleep). Once we deal with the root cause, the seizures go down. Other ideas include:
Elizabeth was never able to sit or stand completely on her own. She has grown increasingly more ‘floppy’ and unable to support or move her body. The challenge here is not just to provide comfort, but to do it in such a way as not to break the bank ($$$$). These ways include:
Elizabeth was a very irritable infant. She cried and wanted to be held a great deal. While she slept well at night, she rarely napped for more than 45 minutes at a time. When she was about 6 months old, she was down to 15 minute naps, from which she would awake screaming and almost inconsolable. Given that our son Jesse is two years older and also needed his share of attention, life was pretty stressful. In retrospect, we know that some of Elizabeth’s irritability was due to gas pains and hard stools, but some was simply a symptom of Tay-Sachs disease which at that time had yet to be diagnosed.
After diagnosis, we were told that nitrazepam, an anti-seizure drug, helped to alleviate irritability when given in low doses. We were, however, unsure as to when we should start using it and the guidance we were given was rather unclear. Elizabeth was 18 months when we did start giving it to her and the result was a very definite improvement. It was as if the connections in her brain had been cleared. We were back where we were a few months before and Elizabeth was brighter, happier and more responsive than she had been for some time. Our great regret is that we hadn’t tried it earlier, so you might think about:
One of the important ways to keep a child comfortable is to keep their skin healthy. Although we’ve managed to avoid them, pressure sores can be a problem with largely immobile children. One of the basic ways we have managed to avoid skin problems is by making sure our child is well nourished and given enough fluids. Other ways are:
Everybody’s heard of some cure for hiccups. For a healthy person, our surefire method is to hold your arms straight up (thereby stretching the diaphragm) and have someone feed you a glass of water. With this in mind, we tried a variation which works quite well for Elizabeth:
Just after Elizabeth turned 3, I read a book by Tom Harpur called The Uncommon Touch. It looks at the roots of spiritual healing and then focuses on the modern day technique of Therapeutic Touch, which is used by over 30,000 nurses in North America. Therapeutic Touch stems from the ancient practice of the laying-on of hands. It is a ‘consciously directed process of energy exchange during which the practitioner uses the hands as a focus for facilitating healing’. It was developed by a nurse, Dolores Krieger, and is used in many hospitals. It basically involves centering or focusing oneself and then moving one’s hands in graceful, sweeping motions a few inches from a person’s body in order to scan the person’s energy flow and restore order and balance to the diseased system. Therapeutic Touch seems to promote a person’s natural healing ability, quickly elicit a relaxation response and reduce anxiety.
Now, I am a non-religious, not particularly spiritual person who jokingly refers to the five minutes that I spend twice a day doing therapeutic touch on Elizabeth as hocus-pocus. But it does seem to help. Since I started doing Therapeutic Touch, our use of pain medication (Tylenol, codeine) has gone down. Elizabeth is more relaxed and better able to fight off infections. I don’t know if it would work for everyone, but as well as being helpful, it’s really pleasant time for Elizabeth and me.
More information can be obtained in Ontario from The Therapeutic Touch Network, phone #905-454-2688.
Another family we know used acupuncture and chinese herb treatments with their child who did quite well. Like us, they also used Western medicine such as anti-biotics, chloral-hydrate and oxygen. They call it the best of the East and the best of the West, highlighting that different approaches to medicine and health can work very together.
Given our natures and the fact that we have two other children, we have taken Elizabeth on quite a few day trips and vacations. Early on, it didn’t pose much of a problem, but it has since grown more complicated. Some things that have made things go more smoothly are:
Odds & Ends
In no particular order, here is some helpful information: