Psychological and Social issues

The Home Care Book

A P ARENT ’S G UIDE T O C ARING F OR C HILDREN W ITH P ROGRESSIVE N EUROLOGICAL D ISEASES

JEANNE M. B ORFITZ , RN,C; FNP EREDITHM M ARGOLIS , P H .D

A Publication of: National Tay-Sachs & Allied Diseases Association, Inc. The Home Care Book

A P ARENT ’S G UIDE T O C ARING F OR C HILDREN W ITH P ROGRESSIVE N EUROLOGICAL D ISEASES

JEANNE M. B ORFITZ , RN,C; FNP EREDITHM M ARGOLIS , P H .D

A Publication of: National Tay-Sachs & Allied Diseases Association, Inc. 2001 Beacon Street Brookline, MA 02146 (617) 277-4463 TABLE OF C ONTENTS

INTRODUCTION ...... I Feeding Difficulties G.E. Reflux A WORD ABOUT FORMAT ...... III Nasogastric and Gastrostomy Tubes Tube Feedings ACKNOWLEDGEMENTS ...... V Constipation Administering Medications PART ONE PSYCHOLOGICAL AND ORTHOPEDICS ...... 24 SOCIAL ISSUES FACING Common Orthopedic Problems PARENTS AND THE FAMILY Hip Problems COPING ...... 1 Contractures MARITAL ISSUES ...... 4 Scoliosis SIBLING ISSUES...... 6 Physical Therapy COMMUNICATING WITH HEALTH Dealing with Special Equipment CARE PROFESSIONALS ...... 7 Wheelchairs, Ramps and Car seats WHEN YOU NEED OUTSIDE HELP ...... 8 FACING DEATH...... 11 NEUROLOGY...... 27 GUILT ...... 12 Seizures Dealing with fevers PART TWO Irritability: Comforting MEDICAL ASPECTS OF HOME CARE Your Child HEENT: HEAD, EYES, Sleeping Problems EARS, NOSE, THROAT ...... 13 Head DERMATOLOGY ...... 29 Eyes Good Skin Care: Avoiding Problems Blindness Adapting the Home for Bathing Eye Infections BEHAVIOR: STIMULATION, Ears PLAY, COMMUNICATION...... 31 Hearing Loss Visual Stimulation Ear Infections Auditory Stimulation Nose Olfactory Stimulation Throat Tactile Stimulation Dental Care Vestibular Stimulation (movement) Communicating with your Child RESPIRATORY SYSTEM ...... 16 Common Respiratory Problems APPENDIX Asthma/Broncho spasm GLOSSARY...... 35 Wheezing MEDICAL ABBREVIATIONS ...... 36 Pneumonia SPECIAL EQUIPMENT ...... 36 Updraft and Suction Machines ABOUT NATIONAL TAY-SACHS & ALLIED DISEASES ASSOCIATION, INC...... 37 GASTROINTESTINAL SYSTEM ...... 18 ADDITIONAL RESOURCES ...... 38 Feeding and Nutrition Positions for Feeding

Photo by Ellen Augarten I NTRODUCTION

PARENTS: You are strongly encouraged to read the introductory This book was made possible through a grant from: sections before proceeding with the main parts of the text. RONALD MC DONALD CHILDREN’S CHARITIES This handbook is a compilation of suggestions, ideas, knowledge, and even and a donation by the: wisdom, generated by a diverse group of individuals who have one thing in ZACHARY EISENSTOCK common: by necessity, each has become an expert in the care of their child MEMORIAL FUND with a progressive neurological disease. “we touch We view this publication as a forum for sharing information and exploring For comments or each other, options. In addition to providing pragmatic advice based on direct experience, suggestions please contact: and teach NATIONAL TAY-SACHS & ALLIED it is meant to give parents new ways to think for themselves and to use their DISEASES ASSOCIATION, INC. own creativity, ingenuity and assertiveness. It is meant, as well, to be seen as a each other. 2001 BEACON STREET work-in-progress. The pages that follow represent a beginning. We hope, over BROOKLINE, MA 02146 Part of the time, to add new insights and generate new ideas. To do so effectively we look (617) 277-4463 healing is (617) 277-0134 FAX to you for reactions and suggestions. This care manual is intended to offer some practical assistance for those who to share.” The Home Care Book Committee: have chosen to care for their children at home. It is written for parents who are Lynn Murphy ready to deal with explicit information and are seeking answers to the ques- Project Coordinator tion: “How do I manage all of this?” Meredith Margolis Author of Psycho-Social section We are aware that some of the information may be difficult to read. You may not be ready to know “too much.” You may find that it is easier to skip around Jeanne Borfitz Author of Medical section and read just the information that pertains to your child “now.” It is important to recognize how much you are ready for and to proceed in a way that is ... all parents of affected children. comfortable for you. Editors: Debra R. Gutter, MSW “We were given names of parents in the area who were caring for children Sedra Schiffman with this type of disease. In the beginning we rejected this type of support. Rhoda Weiner We were in a state of disbelief. We did not know what the future would hold Design By: and we were afraid to talk with others who knew too much.” Essinger Design Associates Newton, MA I I NTRODUCTION A W ORD ABOUT FORMAT

The information in this book is not intended to be “disease specific,” but All of the information contained within this publication has been contributed rather to focus on the wide spectrum of problems affecting children with any by parents who have lived the day-to-day experience of caring for a seriously of the progressive neurological diseases. Although it is written by National ill child at home. Our primary goal in organizing the material was to make it Tay-Sachs & Allied Diseases, Inc. parents, it can be a useful resource for any readily accessible and understandable to other parents. parent caring for a neurologically impaired child. While much of the informa- In these pages we discuss both the medical issues you may face in caring for tion is written with the younger child in mind, some can be applied to the your child as well as the psychological and social aspects of this experience. older child or adult as well. In addition, it is important to keep in mind that the The medical section is presented in a “systems” format, according to the age of onset, progression and life expectancy in different progressive neuro- body system one is dealing with. This was done to make it easier for parents logical diseases vary significantly. Your child may never reach or may have to target the specific information they may need at any one time. The never reached the stages that are discussed. Therefore, some of the suggestions “cardiac” system was purposely excluded as heart problems do not may not be applicable to your child’s condition. commonly affect our children. This publication is not a substitute for professional medical care. It is meant to Where pertinent, we have included a sampling of anecdotal information and augment the information, guidance and care you and your child receive from suggestions from parents about specific treatments, medications and equip- your physician and other health care professionals. Ultimately, the most ment they have found to be particularly useful. We do not endorse any important goal of this publication is to let parents know that they are not alone. individual product or procedure and are aware that these suggestions repre- If we can all somehow reach out and help one another, then we are doing sent only a small fraction of the range of therapeutic options that are something significantly greater than surviving our own tragedy. available. The health care professionals with whom you work will help “Isolation is one ‘condition’ that we can do something about.” guide you in developing an appropriate treatment plan for your child.

We can also be comforted by the fact that through this experience of helping one another, our children have made a major contribution. This is part of their legacy.

II III A W ORD ABOUT FORMAT A CKNOWLEDGEMENTS

Throughout the text, you will notice that we have also interspersed personal We would like to thank everyone who made this book possible. Many quotes from individual parents. We do so in an effort to explore the wide thanks to the wonderful nursing, recreation, occupational therapy, and phys- spectrum of thoughts, feelings and reactions surrounding the experience of ical therapy staff at Northampton Nursing Home for your input. To Allen C. living with and caring for a child with a progressive neurological disease. Crocker, M.D., thank you for your time in reviewing this material as well as Each individual handles his or her situation in a unique and personal way. your valuable comments and suggestions.

We as parents need the freedom to feel our feelings and register our reac- Most of all, we would like to thank all of the NTSAD parents who tions. By reading other parents’ diverse reactions, we hope you will come to responded to our questionnaire. Your input was invaluable to the writing of understand that there is no right way or wrong way to handle this challenge this book. We are indebted to you for your honesty in sharing your personal that life has set before you—-just your way. stories and including your practical knowledge and experiences. While, by necessity, some of the material presented in this publication is technical in nature, we have attempted within the text to define and demys- tify the medical terminology. In addition, we have included an Appendix with a Glossary, a list of Medical Abbreviations, and a Resource List, all of which we hope will further assist you in understanding and utilizing the information provided.

Note: The names of all our children in this publication have been changed to maintain confidentiality.

IV V RESPITE CARE: WHEN A FEW Coping “Karen’s life was far from normal in HOURS ARE NOT ENOUGH Caring for a child with a fatal the number of her days or the extent progressive neurological disease of her abilities. Yet I have often said Sometimes, however, getting away she introduced me to some of the is an immense challenge which for an hour or two is simply not most exceptional people I have ever requires energy, ingenuity and all enough. At these times, respite care known. They, and she, enriched my is a good option to consider. the coping skills one can muster. life in many ways.” For many parents, as painful as the Generally, respite care is care WHO TAKES CARE OF THE child’s eventual death is, the various provided for your child outside of steps along the journey—from CARETAKER WHEN THE CARETAKER’S TAKING CARE? your home for periods ranging hearing the initial diagnosis to from several days to several weeks. watching the child’s inevitable Probably the most important thing Today, some communities have a part one decline—can be equally difficult. to remember when trying to cope with the care of your child is that nursing facilities that can provide It is probably true that you and you need to take care of yourself, respite care, but it is best to check Psychological and Social those close to you will “never be 1 too. Your sick child will not benefit out the availability of these services the same” after going through Issues Facing Parents and from having an exhausted, burned- before you actually need them. If this experience. It is also true that out parent; nor will the other you have the choice of more than the Family this does not have to be a solely members of your family. negative change. Positive changes one facility, select the place that So, what should you do? On a can and do result from extra- “feels best” to you. If you are ordinary situations. regular basis make some time just for YOU. It doesn’t matter how having difficulty in locating a much time you have. Even if it’s nursing facility that offers respite just a few hours each week, care, contact your doctor, Visiting “Parenting a special-needs child can be, getting away from your responsi- Nurse Association, local hospice, bilities at home will replenish your or other parents in your area. at times, an almost insurmountable energies. Do something that you enjoy: go for a walk, go to a And, if no formal facility exists in challenge, and the strain of caring for movie, have lunch with a friend. your community, don’t lose heart. You will be amazed at how revi- Several families have been able to the child at home is difficult for anyone, talized you will feel after such a develop “creative and informal” simple activity, and how much ways of finding respite care. We other than the parents, to realize.” better you’ll be able to cope with have heard of groups of families your situation when you return. forming to help parents take care of their special children. Relatives may also be able to lend an occa- sional hand.

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RELATING: YOU TO OTHERS “Respite is like a shot of of caring for the child at home is “Kate loves hard surfaces therapy to be extremely helpful at MARITAL ISSUES AND OTHERS TO YOU adrenalin. It allows you to regain difficult for anyone, other than the (like my parents’ kitchen table). The some point (or several points) in Any traumatic experience which your energy so that you can get Many, if not most, relationships parents, to realize.” smooth surface enables her to their child’s life. impacts on the two members of a undergo a significant change as a through all of this.” “Due to the level of involvement manipulate herself better. My Whatever avenue or avenues you couple can affect the marital rela- result of the birth of a child with a Having located a place or source for Michael’s care required of us, we parents will sit on either end of the choose to take, recognizing when tionship. While, in some cases, progressive neurological disease. respite care, the next step is to make kept a low profile. Many of our rela- kitchen table (the long way). They you’re feeling overwhelmed and sharing the tragedy of having a The reality is that caring for your the transition as easy as possible for tionships were cut off, as a result of put Kate on her stomach. They put seeking appropriate help is one of child with a serious illness can sick child is an extremely time- you and your child. One parent our own doing or the other persons their hands open on the bottom of the most important coping mecha- bring a couple closer together, more consuming activity, and friends and suggests writing a “Care Plan” to involved. Learning to deal with her feet and she will push off them nisms you can develop. often the experience places a signif- family may not be able to under- give to those who are temporarily people including friends’ and fami- and repeat this until she ends up at icant strain on the marriage. stand the scope of the demands that “I spoke many times with a social caring for your child. This Plan lies’ reactions to his ever changing the other end of the table. Then she Problems present in the marriage have been placed on you. Many worker from Boston Floating might include: pertinent medical and deteriorating condition was repeats the other way.” before the birth of a child with a people may seem at a loss for words. Hospital. He was very supportive. information; instructions regarding painful for us, as parents.” progressive neurological disease You, too, may feel uncomfortable WHEN YOU’RE FEELING He phoned often and when I brought playing, feeding, comforting, may well be exacerbated with the sharing what you and your child are “As Michael’s situation worsened, OVERWHELMED Tim to Boston every few months, we sleeping; and helpful hints for added burdens on the family. going through. As a result, you may we were fortunate to have a few Even with the most supportive would speak at length. [Tim’s sister] solving specific problems. If find yourself cut off from the support friends and relatives that were family and friends, sometimes you saw a child psychologist after the One common source of marital possible, use a computer to record of others around you. supportive and were not afraid to will feel overwhelmed. Caring for a death of her brother to make sure difficulties is anger. It is a natural your Plan so that you can update discuss or deal with our situation.” that she was dealing with it all. I reaction for parents of a child with Experience shows, however, that child with a progressive neurolog- it easily. most certainly believe that this type a progressive neurological disease families who stay involved with the For many families, grandparents are ical disease is stressful, emotionally “When I first decided to leave David of support is needed. We all need to feel angry: angry at God, angry outside world seem to fare better an invaluable resource. While painful, physically exhausting, and at a nursing home for extended help at some time or another.” at the doctors, angry at just about than those who isolate themselves. If grandparents, too, are mourning the sometimes, just overwhelming. At respite, I knew that giving a report anyone. at all possible, try to maintain your loss of their dreams, many have those hard-to-cope times, it is espe- “It was always therapeutic for to a nurse and filling out some outside connections. Keep in mind found that helping their own chil- cially helpful to be able to talk to a me to talk to another parent in the “I didn’t feel guilty about our general information forms were not that those around you may really dren through this difficult time has supportive listener. Good listeners Parent Peer Group of NTSAD. So daughter—I felt angry. I waited enough for me. I had to know that want to help but simply may not made them feel useful and needed. can be found in many places. Some many times we would be faced with until I was married to have a child certain crucial information was know how or what to do. You will When grandparents are able to parents find it particularly making a decision and would feel (unlike all of my siblings.) I planned accessible to all who cared for my probably be able to sense who spend some time taking care of their comforting to speak to other like we had virtually no guidance to have this child. I was going to be child. Only then could I feel among your friends and family is sick grandchild, parents are better parents. They will tell you that it’s from the doctors. It was at these the best mother in the world—and I comfortable about leaving. With this able to be there for you; don’t be able to use that time to be together simply a relief to be able to vent times that discussing other parents’ felt cheated. I also became angry at in mind, I developed the Parent afraid to reach out and give them a or to be with their other children. If your feelings to someone who solutions to similar problems was every healthy child’s parents.” Written Care Plan.” chance to help. you have parents or in-laws nearby, knows exactly what you’re going particularly helpful.” Sometimes this anger becomes and they are willing to be involved, through. Other parents seek profes- “Parenting a special-needs child misplaced and is taken out on those call on them. They can be a sional counseling. In fact, many can be, at times, an almost insur- closest at hand; understandably, that wonderful source of support to you. parents have found mountable challenge, and the strain closest person may well be the

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spouse. It is important to keep in “There are times when I want my At the same time, each may want to SIBLING ISSUES mind that the anger is often a reac- husband to leave because rather recognize that some of your Caring for a child with a progres- tion to the loss, or the impending than offer me support, he adds to my “comforting needs” may best be sive neurological disease requires loss, of the child. If each member of pain. I feel like I’m going through met outside of the marriage. Family immense time and energy. If you the couple can recognize the under- this alone.” members, friends, clergy, coun- have other children as well, life lying feelings of frustration and Finally, numerous couples express selors, or other parents can be an often feels like a juggling act with helplessness, and hear past the concern over their sexual relation- alternative source of support and too many balls in the air. Many anger, they may be able to work ship. The emotional pulls and caring during this stressful time. parents worry about the toll that toward pulling together rather than physical demands of caring for a Parents must also respect each their sick child’s condition will take tearing each other apart. seriously ill child over an extended other’s differences and recognize on their healthy children. Surely “I sometimes have to remind myself period of time often impact on a that no two people mourn in the they will be affected by this experi- that despite everything that is couple’s desire or ability to main- same way. While you may feel ence just as you will be. However, happening to us right now, we still tain their sexual relationship. better being very much involved in with guidance and love, many fami- have each other. That’s why we Sometimes just recognizing that your child’s care, your partner may lies find that their other children married in the first place—to be this is a common reaction to partic- need to be more removed. emerge with a sensitivity and caring together.” ularly stressful situations can help There is no right way to deal with a about others that makes them very special indeed. Disappointment and unmet expecta- couples in understanding and tragedy such as this, and while you tions are another cause for strain in dealing with these issues. may not always be “in sync,” if you Based on their own experiences, the marital relationship. Sometimes “A wedge seemed to come between talk about your feelings and parents of children with neuro- ■ Try to reassure your healthy chil- Sachs disease. The healthy son felt one parent may feel that the other my husband and me, come bedtime. concerns openly and explore solu- degenerative diseases have offered dren that they are OK: they cannot that he was always getting in “just can’t deal” with a seriously My thoughts focused only on my ill tions together, you could emerge some suggestions regarding the care “catch” this illness like other trouble, while Andrew was getting ill child. She or he may feel over- son and I could not seem to enjoy from this experience with a of siblings. stronger, more understanding rela- diseases; and, equally important, so much more attention and never burdened and abandoned. The my husband’s sexual advances.” ■ Try to include your healthy chil- tionship. they had no role in causing it, did anything wrong. Andrew’s unfortunate reality is that both dren in what’s happening. When “Laura was up a lot in the middle of despite any angry feelings they mother’s solution to this problem parents are hurting, leaving limited “How do I cope? I talk and feasible, even allow them to the night. I would get extremely tired may have had towards their was to “admonish” Andrew for energy available for comforting talk and talk about Sandra and participate in their sibling’s care. and lost interest in lovemaking.” sibling. doing things such as coughing or each other. When one needs my feelings to any and everyone ■ Be honest and answer questions ■ “eating” too rapidly through his comforting the most, the other may It seems that the best way to protect who’s interested.” Resist temptations to “deify” your your marriage during this difficult directly. If you are comfortable sick child because he or she never G-tube. The sibling rivalry died be least able to “be there.” “The greatest preventative measure time is to make every effort to keep talking about your sick child and “misbehaves” as your healthy down when her healthy son felt on a “When we found out about against marital stress, for us, was to the lines of communication open. his or her illness, chances are children sometimes do. more equal level with his brother.” Elizabeth he dove head first into talk, talk some more, hug, and every This means that each of you is free your other children will respond Although your time and energy are drug and alcohol abuse. I took once in awhile, have a really good, “One parent told me about her to share feelings, thoughts or similarly. at a premium, it is important to care of everything with little finan- earth-shattering cry.” healthy son and her son with Tay- concerns without fear of blame or remember that your other children cial support from him and no of overwhelming your partner. emotional support.”

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need you too. They need to feel about the medical issues that arise of these progressive neurological we could relax a bit and really listen that such a decision was made working with are there to help you free to be themselves and not feel will ultimately lead to better care diseases are so rare that your doctor better since we weren’t so intent on without my consent.” care for your child. If you feel that constantly pressured to be “good” for your child, as well as help you may not have seen or treated a case memorizing every word. This tech- One problem area that can arise you are spending too much time because Mom or Dad has all that he to feel more in control. So to begin before. Ultimately, the best care nique worked so well for us that we between parents and health care fighting, or that you do not have the or she can handle. Open communi- with, you will want to choose a becomes a collaborative effort got in the habit of taking a recorder professionals relates to the terminal control over your child’s care that cation is especially important now doctor with whom you feel between the family and the medical along on most doctor’s visits.” nature of these diseases. Most you want, perhaps it is time to between you and your other chidren. comfortable. professionals. doctors and other medical workers search for other medical personnel. FORGING A PARTNERSHIP Having caring physicians and other The reality of these diseases is “One of the first things I did after HANDLING COMPLEX INFORMATION are used to aggressively treating that sometimes you may not be Kathy was diagnosed was to find a One area of extreme importance in any medical problems that occur. health care professionals “in your spending as much time with your new pediatrician, someone I felt I In the course of caring for your maintaining an effective working For many parents, however, the corner” can make a world of differ- healthy children as you are with could talk to and who would be child, you may well be learning to relationship between you and your major focus is not to prolong life ence. your sick child. Give your well there to help us through. I actually perform procedures that are new to health care provider is ensuring that but to make their child’s days as “If you ever need to talk to your child the freedom to discuss his or called and interviewed several you. Make sure that you understand you are involved in the decision- comfortable as possible. Sometimes doctor, stand between him and the her feelings about this and other before I made my choice. I was very the instructions you are being given making process regarding your it is hard for health care profes- door. If he tries to walk around you, issues. And give yourself the straightforward about my needs and regarding your child’s care. If you child’s care. Many medical prob- sionals to “switch gears” and take change doctors.” freedom to ask for help if you feel expectations. Not only did I find a are unsure, don’t hesitate to ask the lems can be handled very an approach more focused on you need it. Many families find that good doctor, the experience was a health care provider to explain or differently, depending on your comfort than cure. Open and When You Need demonstrate again. Some parents feelings and those of the health professional counseling by a family very empowering one for me. I felt I ongoing communication about this Outside Help therapist during this stressful time had taken control in what up until find it quite helpful to take along a care professionals with whom you issue is important. tape recorder during doctor’s visits are working. It will empower you Caring for your child at home can can be an important support in then had seemed a totally out-of- “Sara had gotten to the point where so that they can refer to the taped to be an active participant in the be a rewarding experience, assuring helping you and your children deal control situation.” she had a lot of mucus in her throat instructions later on. decisions that are being made and you that your child is living the best with the loss of a child or a sibling most of the time. One of her nurses ESTABLISHING A GOOD may well help you later on with the life possible under the circum- as well as the loss of a more “Very little information was given to kept aggressively suctioning her to WORKING RELATIONSHIP reassurance that you did the very stances. It can also, however, be “normal” family life. me at the time of the initial diag- the point where she would bleed, best you could do for your child. demanding, stressful, and emotion- What does a good working nosis. In fact, the information that moan, and try her best to turn away. ally draining. There probably will Communicating With relationship mean? It means, for was available was so involved that I “I had a very serious problem at Despite my protests, the nurse be times when you feel that you Health Proffesionals example, that you feel free to ask had to use a medical dictionary to one point. The doctor had ordered, continued, explaining that she simply cannot care for your child your doctor for the information you understand its contents.” without my knowledge or consent, needed to suction like that to prevent Establishing a good working rela- need in terms that you can under- on your own. At these times, “When we received the initial diag- to withhold food from my son. It more serious illnesses. It wasn’t until tionship with the health care stand. It means, as well, that you several options may be available to nosis we were too stunned to ask the seems that Jimmy had thrush, which I finally asked Sara’s doctor to write professionals who are involved in feel free to tell your doctor what you to help “lighten the load.” right questions or even remember had been overlooked. The doctor it in her records that this nurse was your child’s care is essential if you you know about your child. In Some of these include: respite care exactly what was said. We called thought that Jimmy had lost his comfortable with suctioning Sara are to manage your child’s illness reality, you are the expert on your (see Coping), home health care, later that evening and asked to have ability to swallow and ‘thought it less frequently, and more gently.” effectively. Being able to talk child; no one knows more about best just to let him go.’ I demanded hospice, and placement. (Consult another conference, this time Keep in mind that the doctors and frankly with your child’s doctor your child than you. In fact, many the doctor return to the hospital to Additional Resources Section in the bringing a tape recorder. We felt that other health professionals you are rescind that order. I was outraged Appendix.)

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HOME HEALTH CARE “We had nursing care during the Josh’s life. I am truly grateful to an easy one, but sometimes it is Home health care aides can help in day so that we both could work. them for what they did.” simply the best one for a family. numerous ways. A health care aide Having nursing care for one night a STATE FUNDED PROGRAMS “I just couldn’t handle it anymore. can free you to do the things you week was a blessing for us, though. The strains on our marriage and life cannot get to when you are caring Even though our insurance provided Many states have specialized were unbearable. I’d had enough.” for your child. An aide can provide for more coverage, we felt that we programs in place to help families a “mini-medical check-up” in your wanted some family time without with children like ours, so don’t “For me, placement has allowed me home. No matter how well you anyone else around. Also, we forget to check into this possibility to get on with my life. I have recov- know your own child, there are wanted to take care of Andy as well. Different states provide ered from the strain of caring for times when a nurse or other health ourselves.” different benefits, but many can him at home.” care professional may detect a assist you in getting low-cost help “We have the ideal set-up. The HOSPICE AND OTHER specific medical problem and may in your home, or even providing nursing home is near my home and OUTREACH SERVICES be able to suggest helpful solutions. respite care at no charge. If dealing job. It has become a second home For some parents, just having an Your local hospice program or with bureaucracies sounds like just for the rest of the family and the staff experienced health care profes- Visiting Nurse Association may another headache for you right now, have become our friends. And the sional in the home alleviates some also be able to provide health care perhaps a friend can help. So many time I spend with him now is just in of the stress, allowing them to feel assistance in your home. The times others ask us if there is holding him and loving him.” mission of these organizations is to more comfortable and confident in anything they can do for us. What a If you are considering placement as give help and support to families When evaluating the alternatives that you can work out a solution caring for their child. valuable gift it would be for the best alternative for you and your coping with terminal illness. Again, that are available to you, it is impor- that is of mutual benefit for both It should be noted, however, that someone to help by researching child, there are several sources some families welcome this kind of tant to check whatever insurance you and the insurance company. parents do give up some privacy state-provided services for you. available through NTSAD that may help, and others may not be And by all means, be sure to check coverage you may have. “My insurance provided generous when someone else, anyone else, is help you in making your decision. comfortable with it. with NTSAD. Resource files are Unfortunately, insurance policies hospitalization benefits. Since I was in the home. This is really a It may be especially helpful for you continually being gathered and are sometimes written in such intent upon keeping Maggie at personal preference issue: to some “Hospice and my family cared for to talk to other parents who have updated and are available to any vague or complicated language that home, I asked that instead of paying the benefits of at-home care Josh. We were assigned caregivers placed their children. Members of interested parents. it is difficult to know exactly what exorbitant hospital fees, would they outweigh the loss of privacy; for for four hours a day, two days a the NTSAD Parent Peer Group help might be provided. Or, as is week. They were trained profes- consider reimbursing some home others, it is preferable to take care PLACEMENT (PPG) make themselves available often the case, a policy may provide care? After numerous discussions, of the child themselves, rather than sionals who took care of Josh. They to talk to other families of children There may come a time when you for quite extensive hospital stays the insurance people finally realized work with another caretaker. would run and pick up prescriptions, with progressive neurological but have no specific provisions for run to the ER with me and would consider placing your child in a that it would be cheaper for them if “Our home aide was a life-saver for diseases. These PPG members are home or hospice care. If you are assist any way they could. They nursing facility, particularly if you Maggie was at home rather than in me. She was so helpful. Sometimes simply parents like you who have faced with either of these scenarios, helped us with the funeral arrange- have no insurance covering at-home a hospital. A side benefit, of course, what was most important was that lived through or are currently it is important for you to sit down ments for Josh and have continued help, and few other resources or was that we would be happier too. she just assisted with other tasks going through a similar situation. with an executive from your insur- to support our family even today. alternatives are available to you. They agreed to pay for as much around the house so I could care for In talking with other parents, you ance company and talk frankly They became our friends, a part of The decision to place a child is not home nursing care as was needed.” Ellen undisturbed.” can express your concerns and about what lies ahead. It is possible

9 10 Psychological & Social issues Psychological & Social issues

questions and learn from their expe- rial service and the people you It is extremely difficult to make the “I have discussed the DNR with prolong the child’s life. Mercifully, with my thoughts. I’ve stopped riences. They are an invaluable want to be involved can be made in decision to use “no heroic efforts” both her pediatrician and neurolo- most such deaths are as peaceful doing that now. But if I could go source of information as well as advance and will save you the to save your child. Even the deci- gist. I don’t know how we’ll feel as we would wish for our children, back, I would have stopped all understanding. added burden of taking care of sion to do everything possible can when actually faced with sticking and for ourselves. unnecessary tests.” “Talking with other families who these arrangements at the time of be a troubling one. In either case, it with this decision. Our feeling in “The last two weeks of Daniel’s life “I felt guilt when I was told that had decided on placement helped your child’s death. Many funeral helps to have the understanding and making this decision is that it were quite different from what we one in four were the odds for my me to feel better about my decision. homes are receptive to making support of the health care profes- is selfish to keep her alive by extra- were accustomed to during any children being born with Niemann- Since I have gone through this expe- advance arrangements and can sionals involved with your child. ordinary means. It is not life to be on other time in his life. Daniel’s diges- Pick Disease. Gregory was my rience, I have been able to help assist you in this effort. And in all cases, it helps to a machine and unaware. When tion completely slowed down to the fourth pregnancy, and my first preg- other families as well.” “I made no funeral arrangements remember that these are very Rebecca’s time comes I pray to have point where he could not digest his nancy resulted in an abortion.” prior to his death. However, personal and individual decisions. the strength to let her go. For what formula or even water for that There are no right or wrong it’s worth, her pediatrician is “Guilt was experienced with hind- Facing Death I wish I had. I let others pick out matter. Danny’s temperature was answers, only your answers, and very good at reminding me of these sight that we failed to fully enjoy her the place for him to be waked, and below normal, approximately 96 more outgoing abilities while she One issue that all parents of a child indeed your answers may change type of decisions and helping me to the people who took care of his degrees, also something we had not still had them.” with a neuro-degenerative disease arrangements were terrible. I guess over time. You are free to change stick with it.” seen before. It was during this must eventually face is the death of your mind and alter your decisions. “Only rarely did I lose patience and I would tell others that, as difficult WHEN THE TIME COMES period that he stopped breathing their child. While scientific infor- as it is to face, you should try to “We have always known that we again, but for the first time we did that is what I regret, that I did at all.” mation and medical experts may make some arrangements or choices would do everything possible to keep Parents are often curious as to just not intervene. Daniel died at 3 a.m. “The guilt I have at times is not describe the typical course of these earlier on.” Tammy alive, never giving up on the how a child does eventually die. exactly two months before his fifth exercising Kathy enough.” diseases, each individual case is While textbooks on these diseases hope that research will result in a birthday.” As is apparent, the guilt reaction different, and it is often difficult to INTERVENTION: state that respiratory infections of treatment becoming available.” runs the gamut from things over accurately predict when or how a DECIDING WHEN AND HOW some kind are usually responsible, “We never discussed this [DNR] Guilt which we truly have no control to child will succumb to his or her At some time in your child’s life many parents report that at some until one day when he was about our simply being less than perfect illness. Several families have faced you may have to confront the point, “my child’s body just seemed When a child is born or diagnosed 21/2, and had to be admitted to the people. Whatever the source, situations where health care profes- issue of the “Do Not Resuscitate” to stop working. He stopped with a devastating disease, it is hospital. He was very weak. He rational or not, guilt can be a heavy sionals have predicted a child’s (DNR) order. Doctors and hospi- breathing and didn’t start again.” natural to want to “look for was having trouble eating and burden and destructive force. Many death as imminent many times (and tals are wary of not doing Interestingly, often a cessation of someone to blame.” Often, it seems, drinking. We knew at this point it parents have mentioned that reading sometimes many years) before the everything possible to save your breathing (apnea) has occurred parents end up either blaming them- was not possible for him to have specific books has been helpful to actual death occurs. child’s life (including performing previously (sometimes numerous selves in some way or feeling bad any quality of life, and it was at this them as they try to deal with and tracheotomies and utilizing venti- times) but there’s “just something about something they did or did not One way to cope with this uncer- point we made that decision. It was work through their feelings of guilt. lators or respirators) unless you different” about the last time. do for their child. tainty is to make as many very difficult to make, and prior Others have been helped by talking have expressly stated that you Perhaps the difference is that the arrangements as you feel comfort- to his getting this sick I would “As his mother, I felt so bad I over their feelings with other do not wish these interventions parent is more ready and less able with ahead of time. Decisions not have been able to make that had put Tommy through so many parents, family members, friends, to be used. willing to intervene at that point to such as burial place, type of memo- decision.” tests. Sometimes I still torture myself clergy, or professional therapists.

11 12 Medical Aspects of Home Care

Note to parents: HEENT: Head, Eyes, side, the ear must be checked peri- You are strongly encouraged to Ears, Nose and Throat odically for pressure areas and read the introductory sections of soreness. Using a sheepskin under this publication before consulting HEAD your child’s head, with a soft cloth the following text. For several reasons, many children over it to absorb moisture is helpful This medical section will hopefully with progressive neurological disor- (see Dermatology section). provide you with additional insights ders have enlarged heads. An When buying clothing for your into caring for your child. Please increased head circumference, child with an enlarged head, you keep in mind that the information which is generally noticed during a should look for shirts, sweaters etc. presented is meant to complement routine physical exam, may be one with large or stretchy neck openings the care and guidance you receive of the first indicators that something to comfortably accomodate the is wrong. from your doctor and health-care head. It may be helpful to remind part two providers. It is not a substitute for The first year and a half of life is well-meaning friends and relatives professional medical attention. the period of greatest head growth, about this special need if they are 2Medical Aspects because the bones of the head are buying gifts for your child. not yet fused. Head growth begins of Home Care to slow down as the sutures (lines EYES between the bones) in the head One of the earliest signs of a poten- begin to fuse and the fontanelles tial neurological problem is the “There were so many things that had (soft spots) close. presence of nystagmus which is a As the size of the cranium grows, rapid, exaggerated, involuntary eye to be done for my child: medications, the head becomes increasingly movement. Although this may be heavy. Holding the child for present to a lesser degree with a tube feedings, exercising and other extended periods becomes tiring healthy newborn, exaggerated because of the weight, especially if movement is an indicator of a necessities of daily care. Then there your child tries to “extend” (arch) neurological abnormality. backwards. You may find yourself The presence of a “cherry red spot” were all the ways in which I could shifting your holding positions and on the retina (back of the eye) is a accommodating for the head size diagnostic sign in Tay-Sachs and ‘stimulate’ him that the therapist when you position your child for sitting or laying down. some of the other progressive suggested. There was so much that I neurological diseases. This can be Some positioning problems exist seen by a trained professional using because of the weight of the head had to do, when all I wanted to do was special equipment for examination and the child’s inability to move. of the eyes. When your child lays on his or her to sit and hold him and love him.”

13 Medical Aspects of Home Care Medical Aspects of Home Care

THROAT Parents may begin to have concerns mucus and a red eye. An eyelash may first present as a conjunctivitis. wash. If you decide to use tooth- Lemon and glycerine swabs or about their child’s vision, especially can also cause redness, so you may Other early signs may be: the Tonsillitis should be considered if paste, use it sparingly. Try to avoid Toothettes are useful in cleaning if the child is not tracking consider looking for a foreign body persistance of a cold with discol- your child is uncomfortable, has a foods with sugar if your child is the palate. If there are dried crusty (following with the eyes) by a irritating the eye before you assume ored nasal mucus for more than one foul odor on his or her breath, or eating by mouth. Dental flossing secretions, you can try a solution certain age, or does not seem to be it is conjunctivitis. Conjunctivitis week; unusual fussiness; loose more pronounced drooling and should also be attempted on a of half hydrogen peroxide and half focusing on a familiar face or may be an early signal of an ear stools; and vomiting. Middle ear fever. When the tonsils are greatly regular basis, if possible. water on a swab to clean the mouth object. infection, so it is important to have infections are treated with oral enlarged, they have the potential If your child is not being fed by and palate. Many children with progressive any persistent redness of the eye antibiotics. for blocking the airway in children mouth, you should regularly Enlargement of the gums (gingival neurological disorders may be visu- evaluated. Be aware that the External infections of the ear canal who have little or no head control. moisten the mouth with a tooth- hypertrophy) may occur if the ally impaired. They may have discharge may be contagious, so (otitis externa) are less serious, but One family used a neck brace to brush or swab. Children often child is on long term Dilantin partial vision or may progress to a precautions need to be taken not to are perhaps more frequent with our support their child’s head during enjoy chewing on the toothbrush; therapy (an anticonvulsant). Gentle complete loss of sight. A test called infect others. Antibiotic drops children because of chronic episodes of tonsillitis. Suctioning if they are not orally fed, this may brushing of the gums is important “Visually Evoked Potential” can or ointment are generally used to drooling which can enter the ear. It may be indicated to help to handle provide the only opportunity to to keep them healthy. help to diagnose the type of visual treat conjunctivitis. is important to change moisture secretions. Tonsillectomy is some- chew. A child can chew on a cold times indicated in cases of impairment your child has. In this EARS laden cloths under the face and ear wet washcloth as well, and the Respiratory System test, brain waves are measured frequently. These infections can be recurring tonsillitis. Tonsillitis may cloth can be rubbed on the gums, while a strobe-type light is flickered Ear infections are very common in painful; they may or may not cause be caused by the streptococcal tongue and teeth which can be COMMON RESPIRATORY in front of the eyes. all children. While these infections fevers. External ear infections are bacteria (“strep” throat) which especially comforting during PROBLEMS are not necessarily more frequent requires antibiotic treatment. treated with ear drops. teething. Stimulating the mouth on Lung problems go hand-and-hand Since it is difficult to measure in our affected children, they do DENTAL CARE a regular basis helps to maintain with the progressive feeding prob- precisely how much vision a child occur and require attention. NOSE has, it is important to provide lots the swallowing reflex, which lems that develop in most children Middle ear infections (otitis media) Regular dental visits and good oral of visual stimulation for your child Because these children cannot blow enables the child to handle his or with neuro-degenerative diseases. have the potential to cause hearing hygiene are very important. Try to (see Stimulation, Play, Commu- their noses, they often have thick her own secretions for as long as As these children begin to have loss. It is especially important to find a dentist in your area who has nication section). Many local dried secretions in the nose. Care- possible. difficulty swallowing, their gagging maintain optimal hearing function experience with special needs voluntary and governmental agen- fully using a nasal or ear syringe One common problem among and coughing can lead to “mini” or in our affected children since so children. The Americal Dental cies provide wonderful services and with some saline solution will help these children is the presence of a major aspirations (inhaling liquid many of them are or become visu- Association may be able to support for visually impaired chil- to remove the discharge. If your high arched palate (roof of the into the lungs) and bronchospasm ally impaired. provide you with a list of dentists dren. You may want to investigate child has persisting nasal drainage mouth). Secretions and food can (spasm in the windpipe). These with this specialty. the services in your area. The more obvious signs of an ear which is discolored, he or she should get caught in this area and are not conditions can cause wheezing, infection are fever and discomfort, be evaluated for an ear infection, as Teeth and gum problems are The most common eye infection in easily removed with the tongue. which is a whistling sound made by but with our children it is helpful to mentioned above. This may also common in children who do not all children is conjunctivitis (“Pink Care should be taken to see that air going through a narrowed learn some of the early signs. As indicate a sinus infection, which is chew or use their oral muscles in a Eye”), which produces yellow the area is clean and moist, as passageway. In addition, these chil- mentioned above, an ear infection treated with an oral antibiotic. normal way. Oral care should dryness can cause fissuring dren often have a lot of mucus in include brushing the teeth at least (cracking) of the palate, which is their lungs resulting in congestion. twice a day with water or mouth painful and can be difficult to heal.

15 16 Medical Aspects of Home Care

Two families mentioned medica- etc., can aggravate your child’s Injury can be caused if the percus- feed or take a bottle; although they tions which they used to help asthma and may further complicate sion (clapping) is done incorrectly, may have difficulty “latching on” decrease the amount of secretions in the picture. so it is necessary to get accurate and the mouth may have to be held in a ® the mouth. They are Scopolamine Of course the main respiratory training, and to feel comfortable certain way to improve the grip. patches (an antihistamine used for condition that is on all of our minds about what you are doing. During It is important to monitor the caloric ® motion sickness) and Artane (an is pneumonia, which can be very this procedure, the child is held and nutritional value of foods that antispasmodic). Ask your health serious in our children. Pneumonia downward and prone (face down). are given orally. Baby food may not care provider about these options. is a lung infection in which sections This position helps to facilitate the provide enough calories as the child Both families felt that these medica- of lung develop severe inflamma- removal of the mucus. Elevating grows. You may have some ques- tions helped a great deal. tion. Children with progressive the foot of the bed (postural tions about your child’s nutritional “John has been like a new person neurological disorders are certainly drainage) to help promote condition: Is my child’s weight since he went on Artane. The side at increased risk for developing drainage of secretions can also be O.K.? Is my child getting enough effect of Artane is dryness of the pneumonia with any respiratory beneficial. The use of an expecto- liquid? How many calories a day ® mouth and it has controlled the infection because of their relative rant (e.g. Robitussin ) was also does my child need? Having a drool and phlegm very effectively.” lack of mobility: they often have recommended to help loosen nutritionist available for periodic secretions in the lungs. Children with neuro-degenerative poor cough reflexes and more consultation is helpful for both oral diseases commonly have a condi- shallow breathing patterns because “When my son is congested, I and tube-fed children. tion called reflux, which causes of decreased activity levels. encourage him to cough by saying Oral feedings can be a joyful, the stomach acids to be regurgi- Swallowing difficulties also make ‘cough’ and then I mimic a cough. special time. This may be a time tated up into the esophagus. them more susceptible to aspiration, He eventually does cough, and I for lots of vocalizations, especially Reflux is a common precipitator which can cause pneumonia. give him lots of positive reinforce- bronchodilator medication (opens that you receive thorough instruc- if your child is very hungry. This of respiratory problems and can “With every cold or flu, with every ment. Who knows if he really gets it. up the airway) in an efficient way. tions on how to suction and that can be a good time to encourage I’d like to think that he does.” also cause wheezing (see Feeding fever, I ask myself, ‘Is this it?’ It is Having a suction machine at home, you are comfortable with the language development by section). on my mind until he is well again.” SUCTION AND UPDRAFT MACHINES although it may actually never be procedure. increasing the child’s under- used, will give you peace of mind standing of certain words and his These children may also develop Physical therapy of the chest (Chest Because of the ongoing potential and may save you an unnecessary, Gastrointestinal or her anticipatory response. For asthma, a condition in which there P.T.), and frequent position changes for respiratory problems in our chil- frantic trip to the emergency room. System example, saying “ready” and is spasm of the airways, causing (e.g. turning side to side in bed and dren, two important pieces of A suction machine is especially repeating the names of favorite wheezing. In addition to the previ- alternating with time sitting in the equipment should be considered for ously mentioned physiologic chair) are beneficial in dealing with convenient to have around during FEEDING AND NUTRITION foods on a consistent basis before the home: a suction machine and an the spoon is introduced are good triggers (reflux, aspiration, etc.), congestion. These measures loosen colds and to suction out the extra Children with progressive neuro- updraft machine. techniques. asthma may also have environ- the chest secretions, thereby stimu- secretions from the back of the logical diseases almost always (See “Stimulation, Play, Communi- mental causes. Typical allergens lating the cough reflex. Chest P.T. If a child does have intermittent cough that your child may have develop difficulty with feeding. cation” section.) such as dust, mold, cats, dogs, involves clapping and vibrating the wheezing, he or she may need an difficulty swallowing. Make sure Initially, they may be able to breast hands over the lobes of the lungs. updraft machine, which delivers

17 18 Medical Aspects of Home Care Medical Aspects of Home Care

Feedings can be incorporated into strollers for transportation for quite for eating. In the neurologically To desensitize a hypersensitive tension) and reflux (see below) and Pain from reflux may result in irri- family meals as a special time for some time, the use of a special seat impaired child, the muscle patterns mouth and thus aid in swallowing, thus further complicate the process. tiability and an increase in muscle all to be together. The children for feeding (e.g., a Tumble Form may never progress past the one family suggests playing inside Very often the feedings are done tension. As mentioned earlier, irri- enjoy hearing familiar voices and seat) in a restaurant may present the sucking stage. and outside the mouth with toys and with the child in our arms. Although tiability (e.g., from hunger) seems ® seeing familiar faces, if they are first situation in which you will face Liquids generally present the first a NUK brush (a dental stimulator this is a very special bonding time, to precipitate reflux, so it is impor- able. This encourages sibling the issue of dealing with “special feeding problem. It can be helpful with soft plastic protrusions on the the increased length of the feedings, tant to anticipate the child’s needs bonding and helps to establish a equipment” in public. (See Dealing to thicken liquids with gelatin, end of it). A Toothette (foam pad on and the child’s increased weight can as much as possible to avoid this sense of “normalcy.” with Special Equipment section.) baby cereal, oatmeal or bran flakes. the end of a stick used for dental combine to make this method of cycle of complications. care) can be used as well. POSITIONING FOR FEEDING “I felt like everyone’s eyes were on Thick It, a commercially available feeding an exhausting experience Although reflux can be somewhat us in a restaurant, saying ‘What’s product used to thicken fluids, may Another family suggests using a for parents. Having another adult controlled by certain medications, Since these children generally do wrong with that child? He should also be used. When eating strained nipple designed for babies with around to help with feedings makes feeding in the upright position, and not progress to sitting up on their be sitting up by now.’ In the begin- foods, the children often will begin cleft palate. This is available free of the process easier. by elevating the head of the bed on own, they are usually fed in our ning, I used to block out their a “chewing” style of eating, again charge from Mead-Johnson. One blocks, it is apt to remain a chronic arms or in their strollers or other stares. It gradually got easier.” because of lack of coordination. It parent suggests a NUK cross-hatch G-E REFLUX problem. Positioning your child on seats that give support to the head is helpful to use a vinyl coated nipple. Cutting off the top or Reflux is a common problem his or her side (sidelying) when in FEEDING DIFFICULTIES and neck. Correct positioning baby spoon to protect your child’s widening the opening of a regular affecting many children (also called bed, is the best bed position to help prevents the children from arching There may be a period of time teeth. Helping them to purse the nipple also works. The child can G.E.R. or gastro-esophegeal reflux). prevent aspiration from reflux or or extending, and helps to “break” during which your child eats and lips by squeezing the cheeks then “suck and chew” out the thick- In reflux, the acid from the stomach vomiting. their high tone (muscle tension), drinks normally. Eventually, prob- together may make chewing and ened liquids. A First Years regurgitates up the esophagus NASOGASTRIC AND enabling them to be more relaxed lems with swallowing affect the swallowing easier. Infa-Feeder was also recommended because of poor muscle tone of the during feeding. GASTROSTOMY TUBES eating process, and taking anything Your child may open his or her for feeding thickened meals. sphincter muscle at the end of the Chronic reflux, exhausting and They need to be fed in an upright by mouth becomes difficult. The mouth more easily if he or she Evenflo makes a similar product. esophagus. difficult feedings, and problems position and the head needs to be in child may start to cough or gag smells the foods first (and you can Invariably, the entire feeding In children with progressive neuro- with dehydration from lack of midline, not extended back, in order during feedings. He or she may also encourage understanding of process eventually becomes very logical disorders, who are already adequate fluid intake are often the to help prevent aspiration and develop a poor suck. He or she may language if you say what the food is time consuming. The children need beginning to have difficulty swal- precipitating factors leading to the reflux. (See G-E Reflux section.) begin to aspirate food which can prior to introducing it). Another to be fed slowly to prevent aspira- lowing liquids, this chronic decision to begin tube feedings. When not being held, your child cause pneumonia, or have “mini” way of getting the mouth open is to tion, and they tend to eat slowly. regurgitation of stomach acids A video esophagram can be a can be propped up to a sitting posi- aspirations which can precipitate apply firm pressure on the corner of Feeding can be very tiring for our further compounds the problem. helpful test in determining whether tion with lots of pillows and stuffed bronchospasm, and cause the jaw and manipulate the area children, and they may need to rest Chronic reflux causes irritation of or not tube feedings are necessary. animals for comfort and support. wheezing. (See Respiratory (also a useful technique for dental during the meal. By experience, we the esophagus resulting in “heart- In this test, an x-ray “video” of the Accepting the fact that your child section.) care). You can encourage chewing have learned that it is also important burn”, an inflammation of the esophagus is taken while the child needs special equipment is a The mouth muscles are designed by placing your hand along the to feed them on time. If the child is esophagus (esophaghitis) or a much is eating. gradual process. Because very for sucking during infancy, and as lower jaw bone and moving it up very hungry, the resulting irritia- more serious problem, ulcers of the young children can “get by” with the child grows, the muscles adapt and down. bility may increase tone (muscle esophagus.

19 20 Medical Aspects of Home Care Medical Aspects of Home Care

There are generally two methods Matthew’s life. Yet the part that was quickly, so it must be monitored for correct caloric and nutritional of tube feeding to choose from. so painful was that we did not want carefully. Another method is called content as the child grows. It is also Some parents choose a naso- to be responsible for killing him, a bolus feeding (a certain amount important to give plenty of “free” gastric tube (N-G tube) in which since feeding Matthew had become going in all at once) in which a water (plain water), and to flush out a tube is inserted into the child’s a risky activity.” 50cc syringe is refilled with food the tube with water after each nose and threaded into the “Feeding had become so difficult; and given evenly over an hour or feeding to prevent blockage. Your stomach. he was losing weight and he had so so. This technique is handy for trav- health care provider can assist you “We chose this option because we many respiratory problems. We eling, or in case of electrical failure. with this information. felt that we did not want to put him prolonged it for as long as we You may choose a traditional Even when a G-tube is placed, it is through the gastrostomy operation could. But once the crisis of the formula or you may make your important to continue some level since his health was so bad.” surgery was over, we knew that we own in a blender from table food. of oral feedings for as long as The advantage of the N-G tube is had made the best decision.” The following recipe was made by possible to maintain the swal- that it is a non-surgical procedure “When my husband and I came to a parent each morning for her lowing ability as well as to and can easily be removed (it is the full realization of what was sixteen month old and used stimulate the sense of taste and usually replaced once a month). going to happen to our son, we throughout the day. The recipe was smell. Considering, however, that The disadvantage is that the N-G decided not to place an in-dwelling given to her by a nurse who oral feedings at this stage may be tube can be irritating to the nose gastrostomy tube. This decision was researched G-tube feedings. very difficult and that adequate and can involve some risk (the tube made in private without counseling It is important that both the non- nutrition is provided by tube feed- may inadvertently slip into the Some parents choose gastrostomy the “button” may have to wait until from anyone. It was made over a commercial and commercial ings, “tastes” of food are sufficient lungs). It needs to be taped to the tube (G-tube) placement, which is a the original incision is healed. period of months, as we witnessed feedings be evaluated periodically to maintain these skills. nose to prevent dislodging. It is surgical incision into the stomach The decision to place a G-tube or the progression of this devastating essential to check for proper place- through the abdomen. In conjunc- use an N-G tube is a big one, as it is disease. We decided that to the best Ingredients Calories Carbohydrate Protein Fat ment prior to starting a feeding by tion with gastrostomy tube often the first major intervention in of our knowledge, when our child 1/2 cup cooked cream of wheat 68 15 2 placing the end of the N-G tube in placement, a fundoplication is our children’s lives. Indeed, some could no longer eat without water to check for air bubbles. If air usually done to help the reflux families may decide not to tube surgical intervention, he would be 1 jar baby beef 100 1 14 5 problem. Fundoplication is a bubbles are present, the tube is in feed at all. One struggles with the permitted to die.” 4 oz. orange juice the lungs. The N-G tube then needs surgical procedure in which the desire to protect the children from 1/2 jar baby carrots 17 3.5 .5 to reinserted properly. While many stomach is “enveloped” around the such intervention, while trying to TUBE FEEDINGS parents learn to handle this proce- end of the esophagus, thus find ways to make them most Most parents use a traditional G- 6 Tbsp. non-fat dry milk 166 23.33 16.22 .37 preventing stomach contents from dure, proper instructions and comfortable. tube pump for the regular feedings 2 slices whole wheat bread 136 30 4 training are very important. You “refluxing” up into the esophagus. “We discussed the idea for months. which can be run by battery for 3 Tbsp. corn syrup 180 45 will want to give yourself a chance A parent may choose a traditional It was a most difficult decision. We some time after being unplugged. A 3 Tbsp. lite corn oil 450 50 to feel comfortable with N-G tube G-tube, made out of silastic or were strongly against medical gravity fed bag can be used for trav- insertion before attempting it on latex, or a “button” which lays flat intervention that would prolong eling; however, there is a danger 1 3/4 cups water – – – – your own. against the abdomen. Insertion of that the formula will go in too

21 22 Medical Aspects of Home Care Medical Aspects of Home Care

Another method of stimulating the It is important to include plenty of It is also not unreasonable to place that your finernail is short before sauce or some other food. The pills that result from high tone (muscle sense of taste is to use a foam swab “bulk” or “roughage” and fluids in the child on the potty chair after performing this procedure. can also be soaked instead of tension) and lack of weight bearing (Toothette) dipped in flavored the diet of an orally fed child. Fresh feeding to take advantage of the Continued severe straining during crushed. Some pills may be diffi- (standing). water. One occupational therapist fruits, vegetables, and grains such gastrocolic reflex, or urge to have a cult to dissolve, so you may have to bowel movements may cause HIP PROBLEMS calls this “gum on a stick” because as bran flakes should be incorpo- bowel movement at this time. Thus hemorrhoids, rectal fissures or even go back to the liquid form. children like to chew on it. rated into the diet. As liquids gravity is allowed to help with this rectal prolapse (where the rectum Consider rectal suppositories as Hip subluxation (partially out of the become more difficult to take, they normal process. The child can be hip socket), which can lead to dislo- CONSTIPATION actually herniates outside the anus). another means of administering can be thickened with bran flakes. held on the potty chair in a slightly It is important to keep constipation medications. Tylenol® (aceta- cation, can occur at an early age. Children with neuro-degenerative If prune juice is used, keep in mind bent forward position. Of course the under control to avoid these compli- minophen) is available by This problem is generally identified disorders eventually develop prob- that it can cause diarrhea when child will enjoy any positive rein- cations. If the rectum does become suppository. Also available are during an infant physical examina- lems with bowel movements. This given in large amounts. forcement after a job well done! inflammed with hemorrhoids or glycerine suppositories for consti- tion. To treat this condition, the is due to changes in the nerve cells For G-tube feedings, one parent It is best to try to prevent constipa- fissures, ask your health care pation. Other specific medications child is positioned with the legs of the intestines that result in a recommmends a soy-based tion by the above measures. Once provider for a hemorrhoidal cream mentioned by parents are Tigan®, spread apart or “abducted”. This decrease in gastric motility (move- formula to help with constipation. the child is constipated, there are with steroid (Anusol®-HC) which Phenergan® and Dulcolax®. Ask can be accomplished either manu- ment of the stool through the Another suggests adding one several ways to deal with the provides a soothing, anti-inflamma- your pharmacist if a particular ally by proper holding techniques intestine) and is complicated by the tablespoon of vegetable oil to the problem. Fleets® enemas for chil- tory effect. medication your child needs is (e.g., holding the child with your relative inactivity of our children. food or three tablespoons of wheat dren are good. The containers can available in suppository form. arm between the legs to keep them Certain medications can also aggra- ADMINISTERING MEDICATIONS apart), by pillows wedged between germ. Ensure is a formula be cleaned and reused by making When giving medication via vate the problem. the legs, or by the use of a special enriched with fiber. Natural laxa- your own solution of water and Medications for children generally feeding tube it is important not to ® brace. Dealing with special prob- It is not uncommon for a child to go tives, such as Metamucil , are liquid soap (one tablespoon soap come prepared as liquids for oral mix it with the feeding. If the lems like these has prompted several days without a bowel move- generally better for the child as and fill with water). Mineral oil use. To make them more palatable, feeding has to be stopped for some several parents to explore unique ment and not feel uncomfortable. they provide bulk which helps to enemas (the plastic containers can they are generally sweetened, artifi- reason or another problem occurs, and creative solutions. However, severe constipation can stimulate the bowel. One family also be cleaned and refilled with cially colored, and have alcohol you will not know how much of the cause a great deal of discomfort, recommends Malt-Supex powder mineral oil) or glycerine supposito- added. You may prefer to shop medication was absorbed. It is also “My child was given a brace and measures should be taken to be added to each feeding. ries can be used as well. Stool around for over-the-counter medi- important to flush the tube with called a Pavlik Harness ® at six prevent this problem. Even though our children can’t get softeners are also available under cines for children that are prepared water after administering a medica- months of age for his subluxed hip. various names. And stimulating the As it becomes more and more exercise in a traditional way, without sugar or alcohol (e.g., cold tion through the tube to ensure that It strapped over the shoulders, rectal area with a lubricated cotton difficult for your child to accept exercising the arms and legs or pain medications). the whole dose of medication enters attaching to the thighs to hold them swab or thermometer may adequate amounts of fluid by passively (someone else moving the Because of these additives in liquid the stomach. in abduction. The straps dug into encourage a bowel movement. mouth, severe constipation can arms and legs) may be helpful in medications, you might ask about his shoulders, causing discomfort result. Once tube feedings become improving digestion. Massage of Occasionally it is necessary to getting medications prescribed in Orthopedics and abrasions. He cried constantly the main source of nutrition, the the abdomen along the large colon disimpact (manually remove) the pill form. Pills can be crushed with while in it. My mother and I problem with constipation generally (up the right side, across the top, rectum of stool. Use a latex glove a mortar and pestle (found in COMMON ORTHOPEDIC PROBLEMS designed a “vest” that attached to down the left side) may help to with lubricating jelly (or a finger the thighs in a much more comfort- subsides, although this is not gourmet food stores) and given in a Most of the orthopedic problems move the stool along. cot). You will want to make sure able way for my child.” always the case. feeding tube or by mouth in apple that affect our children are those

23 24 Medical Aspects of Home Care Medical Aspects of Home Care

WHEELCHAIRS, Parents should not feel inhibited Various splints can be worn during “A body jacket was just one more needs children is to begin to use include: Snug Seat and Tumble RAMPS, CAR SEATS about designing any equipment that parts of the day or during the night intervention that I could not deal adaptive equipment which finally Form Carrie Seat® and Carrie may make their child more to help prevent the progression of with. I couldn’t imagine him being identifies our children as being Adaptive wheelchairs provide good Rover® (the stroller part). A good comfortable and make life easier. contractures. Ankle-foot orthotics comfortable in that hard plastic. I “different.” For a while, we can head support and can be adjusted to car seat that has been recommended Should you find a helpful and (AFO Splints) can be used to also had trouble with the idea of the get by with traditional car seats the contour of the child’s body, by parents is the Evenflo 7-year creative solution to a problem, prevent foot drop. These splints plastic coming between our bodies and strollers, but eventually, as our providing comfort as well as Car Seat.® helping to slow the progression of please share your experience with generally are made from a thin while we were snuggling. But he children grow, the traditional As the child gets heavier and more orthopedic problems. Some of the NTSAD so that other parents can plastic material, extend over the heel was clearly becoming uncomfort- equipment no longer provides the difficult to carry to and from the smaller wheelchairs, such as the benefit from this information. and foot up to the mid calf and keep able as the scoliosis progressed. support the children need. house, parents may want to Mulholland Travel Chair ®, convert If your child’s hip problem the foot at a 90 degree angle. They The body jacket definitely helped. Here again a parent’s ingenuity consider installing a wheelchair to a car seat which is an added progresses to dislocation, and your are worn over a sock, but you can His scoliosis actually improved and can provide successful solutions. ramp. Because of new accessibility convenience. Other stroller-type child is not walking or standing, it put a sock and shoe over it as well. he is now more comfortable. I just As an interim measure, one parent laws, many carpenters are wheelchairs that convert to car seats may not be a problem. In the Surgery can also be done to correct take it off when I want to snuggle used a “twin” stroller with a front becoming familiar with building absence of weight bearing, there is tight heel cords that can cause the with him.” and back seat. By putting the back foot to point downward. Hand usually no discomfort from a PHYSICAL THERAPY of the front seat down she made a dislocated hip. splints can be worn to help keep the “longer” stroller which worked thumbs abducted (spread apart). The ongoing involvement of a very well for transporting her ® CONTRACTURES Neoprene Sof-Splints allow for physical therapist in your child’s child. Graco makes a good twin Another common orthopedic normal thumb movement and help care is very helpful in preventing stroller. It can be further adapted if problem is contractures. With to prevent cortical thumb (when the various problems and in monitoring need be. this condition, the joints remain thumb crosses in over the palm). existing ones. Early intervention programs may provide this service. Eventually, however, most chil- slightly flexed (bent) and eventu- SCOLIOSIS dren require special equipment. ally are no longer able to In some states, supportive services Curvature of the spine (scoliosis) such as physical therapy are The first transitional piece of completely return to their normal equipment is usually a wheelchair. position. This condition inevitably can also be attributed to high tone, included within the scope of special which gradually causes the spine to education under Entitlement Law No longer can a parent go to the occurs over time. The best way mall “unnoticed.” This is a big, to prevent or limit contractures is curve towards the area of greater 94-142. Check your state or local tone. If the curve becomes severe, it programs to determine what brave step, but if we are to func- to continue a regular physical tion and be part of the world therapy program which includes can cause discomfort as the hip and services are available in your lower rib cage begin to meet and region. around us, it is one that many of exercising the joints, ligaments us learn to take and somehow and muscles with passive exercise the internal structures become compressed. A body jacket is the DEALING WITH learn to cope with. (See techniques. Some of the common SPECIAL EQUIPMENT Positioning for Feeding section.) contractures are: wrist drop and one answer to help stall the progres- One of the most difficult transitions foot drop, as well as contractures sion of scoliosis and give the child “With our children, we must contin- we must make as parents of special of the knees. relief from discomfort. ually accept with reluctance.”

25 26 Medical Aspects of Home Care Medical Aspects of Home Care

DEALING WITH FEVERS codes for adding access ramps to necessity, expert detectives in fevers is “fanning” the body with a One of the many challenges we It is important not to overstimulate existing structures. Increasingly seeking out valuable resources. And Fevers tend to go quite high in our hand-held fan or piece of paper to must face is how to console an irri- a child if he or she has a tendency attractive and “less obvious” ramps by all means, when friends and children and can accelerate quickly promote evaporation. If you use an table child. The challenge is not to become irritated. For example, are becoming more commonplace. family want to help, let them. In the because of the instability of the electric fan, make sure it is across only in finding ways to console many children get extremely upset While building a ramp can be an end, not only you will get some fever center in the brain. High the room from the child. Keep your child, but also in finding ways when their faces are touched. Loud expensive project, funding assis- much needed assistance, but those fevers need to be treated aggres- your child covered with a sheet or to take care of yourself, as the or sudden noises, although enjoyed tance may be available from who offer that helping hand will sively, because they can precipitate light blanket so as not to chill the child’s irritability can be ongoing by some children, may cause irri- various sources. feel good about being useful. seizures. body too quickly. and very stressful. tability in others. Sometimes a child You need to know how to read a “When my son was in for an asthma can be kept more comfortable with SOME THOUGHTS ABOUT FINDING IRRITABILITY: medications to alleviate discomfort THE NECESSARY RESOURCES TO Neurology glass thermometer. It is best not to COMFORTING YOUR CHILD admission , prior to G-tube insertion, or encourage sleep. HELP MEET THE GROWING NEEDS rely on digital thermometers, as I recall one night that I’ll never One of the common symptoms of IN YOUR FAMILY: SEIZURES they can be inaccurate. Rectal forget. The only thing that would “There were times when I would neurologically impaired children is Many of our children have seizure temperatures are most accurate console him was having me walk him become so frustrated because of the Many of us find it very difficult to irritability. Irritability can be disorders of one kind or another. (three minutes). Axillary (under the up and down the halls. I did this for constant irritability that I would just ask for help. But this is an extraor- caused by neurological immaturity Although undesirable, seizures may arm) temperatures take too long six hours straight, holding him in my resort to giving him a medication for dinary experience we are living or the disease progression itself. It not be as damaging to a child as we (ten minutes). arms with an IV pole in tote.” sleep. Nothing else seemed to work. through, with extraordinary is often compounded by related might expect, as long as the seizure I had to convince myself that this demands placed on our time, energy Always have acetaminophen conditions such as reflux, ortho- Singing, humming, or just the is not prolonged. A prolonged ® was O.K. to do.” and resources. Along the way, we (Tylenol ) on hand for fevers. pedic problems or other sound of a familiar voice can help. seizure can cause anoxia (loss of will all be faced with the challenge Never use aspirin for fevers because discomforts. Your child may Music is soothing also. Sometimes SLEEPING PROBLEMS oxygen) to the brain. of learning new skills; one of those of the risk of Reye’s Syndrome. “grow” out of a stage of extreme the child just needs to hear a contin- Sleeping difficulties are a common challenges may be learning to ask Seizures usually can be controlled Have the acetaminophen available irritability as he or she matures. If uous familiar sound. One thing that problem with these children. They for, or simply to accept, help. with medication. The kind and in both suppository form and liquid irritability is ongoing, and you usually works is motion: holding, may be up several times a night for There are organizations out there amount of medication may change form so that you have options for sense your child is experiencing rocking or walking the child. various reasons: muscle spasms, which are willing to help families over time. An increase in the use. Use the acetaminophen for any discomfort, speak to your health “We had our washing machine in reflux discomfort, nasal conges- with sick children. Indeed, some are frequency or severity of a child’s fever over 102 F. (Ask your health care provider. A specific problem the kitchen. When my son became tion. They may not take naps specifically dedicated to that goal. seizures may be due to a progres- care professional if he or she wants could be identified and corrective fussy, I would put him on top of the during the day. It may not be When you are confronted by a sion of the disease or may indicate it given sooner than this.) Fevers measures taken. For example, washer in his infant seat, and put it possible for your child to “cry special need, research the facilities that the dose of the anticonvulsant under 102 F should be closely esophagitis (inflammation of the on spin. The vibration would always himself to sleep.” As the crying and services available in your area. is not high enough for his or her monitored for sudden elevation. esophagus) caused by reflux can console him.” becomes more intense, muscle Check with the NTSAD office to weight. Optimum doses of medica- With a very high fever, over l04 F, be very uncomfortable for the (If you decide to try this method, tone, reflux or wheezing can see if their resource files can tion can be monitored in part by you can try a sponge bath (not tub child. A fundoplication may make sure that the seat is secured increase causing greater discom- provide any leads. You may want to blood levels drawn on a periodic bath) using tepid water (lukewarm). correct the reflux problem (See G- somehow to the washer and that the fort. Occasionally, just patting the ask other parents for guidance as basis. In some cases, vigorous Lay the moistened cloths on the Tube section.) child is strapped into the seat.) child on the back will work, but well; many of us have become, by efforts to control seizures may not child’s forehead, armpits and groin. be justified. Another method to control high

27 28 Medical Aspects of Home Care Medical Aspects of Home Care

be checked regularly for redness or site and no obvious sore spot, the was also recommended to provide irritation (pressure areas). If a pres- irritation may be a reaction to the tactile stimulation. sure area shows up, the child should material the G-tube is made of. In A regular kitchen sink may be fine be kept off that area until normal this case, it may be worthwhile to for bathing your child for awhile circulation resumes. Any skin consider changing the type of mate- (the height is usually good). You breakdown should be monitored for rial (e.g., latex to silastic). (See may want to consider installing a signs of infection (redness, swelling G-Tube section.) longer sink to accommodate your or discharge). BATHING child as he or she grows. Because our children are in diapers, Bathing is a special time to be with Eventually your child may frequent changing of wet diapers is outgrow any sink, and you will important, both for asthetic reasons your child, although it does require some organization and planning. need to try tub-bathing. Since and because prolonged exposure to lifting a heavy child in and out of moisture can cause rashes. The bathing room (bathroom or kitchen) should be warm. A time a regular bathtub can be difficult, Good skin care around the G-tube should be set aside and things bathroom renovations may (Gastrostomy tube) site is important should be relaxed, without too much become necessary. If financially to help cut down on drainage, interruption (e.g., phone). The feasible, consider installing an prevent infection and to keep the temperature of the bath water should elevated bathtub with a hand held child more comfortable. The area be tested first by you (using your shower. This makes it easier for an can be cleansed with a solution of elbow) to make sure it is not too hot. older child to be bathed in a bath half hydrogen peroxide and half Soap should be used sparingly; too seat, and lifted in and out of the water. This will sanitize the area much can cause skin dryness and bathtub. One family had this type often he or she will have to be our children. Sheep skin pads and Dermatology and help to debride (remove) any itching. Talcum powder should not of tub installed. When it was not picked up and held, rocked or egg crate foam pads also work buildup of secretions. If there is an be used at all, as it can be inhaled being used for the child, a GOOD SKIN CARE: sung to. If sleeping problems well. If your child has difficulty area that looks irritated at the stoma and may cause lung irritation. movable step was placed in front AVOIDING PROBLEMS site (opening for the G-tube to enter of the tub, giving it the appearance are chronic it may be helpful to going back to sleep at night, try to One family recommends using Good skin care is especially impor- the stomach), an application of a of a “Roman Bath.” They also consider giving a sleeping medica- arrange medications and treat- dotted jersey gardening gloves for tant for many reasons. Our children silver nitrate stick will cauterize the installed a sink with a long tion on an occasional basis. ments so that he or she does not handling a slippery child during are relatively immobile and are area, promoting healing. This counter, making an excellent Chloral Hydrate is probably the have to be awakened. If the child bathing. This is especially impor- susceptible to skin breakdown and causes momentary discomfort, but changing table. most useful sleeping medication does wake up, do the diaper tant when taking the child in and pressure sores. Frequent turning and the subsequent healing from the available for our children. change, turning, treatment or out of the tub. The gloves also Good bath supports will make life position changes are essential. Body application should reduce the Some suggestions: Providing a medication during that time, if at provide some nice tactile stimula- easier. When your child is very prominences, such as hips, shoul- drainage at the site. If there is warm, comfortable bed is essential. all possible. tion as you are washing your child. young, you can place him or her ders, shoulder blades, etc., should chronic drainage and redness at the Water mattresses are excellent for Using a Water Pic shower massage on a bath sponge, or a “Tubby”

29 30 Medical Aspects of Home Care Medical Aspects of Home Care

inflatable raft-type cushion. An Sensory stimulation activities are ducing the stimuli. You might want it is too quiet. Provide tapes with sounds your child may enjoy touch a special toy before playing inflatable bath pillow will help to varied and are open to much to try this technique while feeding both soothing and more lively (different animal sounds, ocean with it, or feel an item of clothing support the child’s head. This is creativity, depending entirely on your child. music, depending on the mood you sounds, etc.) before dressing. especially helpful with children your child’s specific situation. are attempting to create. Some chil- VISUAL STIMULATION OLFACTORY STIMULATION Look for things that provide a who have enlarged, heavy heads. Basically, anything that will stimu- dren enjoy sudden, loud noises variety of textures for your child to Rifton makes an excellent bath seat. late your child’s sense of sight, Bright flashing lights, contrasts (dropping pots and pans or The sense of smell can be stimu- touch with his or her fingers or for Columbia makes another. One hearing, taste, smell, and touch, as of lights and darks, intermittent pretending a loud sneeze). This is lated by utilizing vials of food you to rub on the child’s skin. Many family designed their own seat with well as sense of motion, is included introduction of stimuli, and move- one activity that siblings greatly flavorings (peppermint, vanilla, “special” toy catalogs (see PVC piping and lawn chair-type in these activities. The senses ment of stimuli can serve to enjoy helping with. Provide as orange, etc.) or by just having your Resources in Appendix) have toys webbing from a hardware store. should be stimulated whenever encourage vision. many types of sounds as you can child smell the family’s dinner. designed with various textures. You possible, even if there is some Several of the newer baby mobiles think of for variation: music boxes, And, of course, throwing in words can make your own texture board or sensory impairment. We can never Behavior are made with black and white radios, “Walkman-type” tape like “spaghetti sauce” may blanket (or have a special friend or be totally sure of the level of our designs. Black and white are good players (make sure that the volume encourage learning. Likewise, if relative make it for your child). Any children’s vision or hearing impair- STIMULATION, PLAY colors for early visual development; dial is fixed somehow to prevent your child is able to, tastes are rough, smooth, ribbed, rubbery, AND COMMUNICATION ments. then progress to red and other accidental turning up of the encouraged so that the sense of sticky surface will do. You may Families are wonderfully creative in Part of play is also learning. bright and shiny colors. Shiny pom volume), stuffed animals and other smell is connected with the sense of want to experiment with liquids and finding ways of interacting with Although it is difficult to measure poms and pinwheels are great! toys that make sounds. There are taste. Smelling and tasting have the other textures as well (play dough, their children. Since many of our what our children are capable of Shiny or colored tape can be several battery-run musical instru- added benefit of stimulating the finger paint, shaving cream, children are visually impaired, we learning, we shouldn’t assume that applied to less interesting toys and ments (e.g., keyboards) that your appetite and aiding in digestion. pudding!). The possibilities are look for learning or creating ways they are unable to learn. objects to encourage tracking. Toys child can “play” while you place his Note: If your child tends to have endless, and your child will enjoy to encourage stimulation of the with lights, especially flashing or her hands on the instrument. This seizures, you might try to discover the new experience. “Robert has been in an integrated will also encourage “cause and other senses (see Eye section). We program at our public school for lights, are also appealing. if anything in particular seems to One way of encouraging cause and all know what a gift it is to hear our effect” learning. Keep in mind that a few years now. I know that he Toys are available that can be bring them on. Certain colognes, for effect is to provide tactile “cues” for children laugh or see them smile, some children become very irritable has ‘learned’ counting on a adapted with large, easy to operate example, have been known to your child when you or someone and we all will do just about when exposed to loud and sudden number of occasions. When I fold switches so that children with precipitate seizures. else greets him or her. For example, anything to get those responses! noises. Sensory stimulation activi- the laundry with him, I ‘count’ the neurologic impairments can turn ties need to be adapted to each TACTILE STIMULATION help him or her to touch your ring As they get older and the neurolog- socks, undershirts, etc., over and them on and off (Switch toys). child’s needs. or watch as you repeat your name. ical system has a chance to mature, over again. He really seems to Mirrors (made of soft material, not Any physical contact that you have VESTIBULAR our children may become less irri- enjoy this as if he understands glass) can be taped to the bed or If your child is to be away from with your child will stimulate his STIMULATION (MOVEMENT) table and more open to play. The what the numbers mean.” play areas. you (e.g., during hospitalizations or her sense of touch. Providing or respite care) you might consider tactile cues as a part of routine Children generally love to be held, chronically irritated child who hates One way to incorporate “cause and AUDITORY STIMULATION a tape of familiar family voices daily activities allows your child to bounced, walked or rocked. to have his or her face touched, and effect” learning into your child’s singing, laughing and talking, or participate more fully in his or her Sometimes, this is the only thing that has never smiled, may grow out of play is to practice anticipatory skills Our children seem to enjoy lots of the dog barking. There are lots of life. You can have your child hold works with an irritable child (see this behavior. by saying “ready” prior to intro- sounds and will often “complain” if tapes available that have various the toothbrush before mouth care, Irritability section). Some children also

31 32 Medical Aspects of Home Care

enjoy more vigorous movements child to be easily moved and to be “There were so many things that else, we’ll go to great lengths to like swinging or rocking. Twirling “where the action is” if other chil- had to be done for my child: get a laugh from our children. around, however, may upset your dren are around. (You can order a medications, tube feedings, exer- Use every opportunity to look for child because of immaturity of the low, removable base on wheels for cising and other necessities of daily signs of communication from your vestibular system (which gives us a Tumble Form seat.) care. Then there were all the ways child, and make an attempt to rein- our sense of balance). Many parents report that waterbeds in which I could ‘stimulate’ him force any attempt that your child It is important to provide a variety are great and provide a range of that the therapist suggested. There makes to communicate. A signal of “places” in which your child can vestibular bouncing and rolling was so much that I had to do, when may be as simple or subtle as a play and learn. Bean bags are great sensations. Waterbeds can be all I wanted to do was to sit and tense muscle. These signs may because they mold to the child particularly helpful during periods hold him and love him.” initially be random, but if you (and siblings enjoy them as well). of fussiness. Children with poor Each of us and each of our chil- give the signal a meaning, perhaps Hammocks can be hung in a head control need to be watched dren are unique and we need and your child will learn to give it a common room and are easy to take carefully while on a waterbed, deserve the “space” to exercise our meaning as well. down when not in use. Make sure however, to avoid suffocation. own judgments in our own ways. “I would talk a lot to him and that your child is safely secured in Provide cues for your child before reassure him. He responded to me the hammock and that you are COMMUNICATING moving him or her. Say “We’re WITH YOUR CHILD and totally trusted me. He knew my standing next to him or her. getting out of the chair now” and voice, my touch, my smell...as he As parents we are usually very Hammocks provide motion and touch his or her arm. Or say, lost some senses, others would sensitive to a child’s subtle signs. vestibular stimulation. “Ready” or “1-2-3.” This helps become more pronounced...don’t We can tell if the child’s cry is one Corner chairs, which are triangu- your child to be part of the activity. underestimate what they can do of pain or boredom; if the child’s larly shaped, provide good support It also avoids startling the child. –and adapt!” face is showing discomfort or for sitting and can be easily “I always told him what I was hunger. We are the experts in inter- adapted to fit your child. For doing and even though he may not preting the needs of our children. smaller children, a baby carrier have understood me, he knew the Vocalizations from our children are (e.g., Graco Courier Soft Baby routine, he trusted me and little gifts. Smiles and laughs are Carrier) provides good head approaching him this way always even greater gifts. Vocalizations support and enables you to take seemed to minimize his fears and can be encouraged by repeating the your child around with you as you seizures.” work around the house. A carrier sound after it is made by the child. also allows for greater accessibility One thing that many of us have Encouraging laughter is usually outdoors. Prone standers (e.g., by learned through the years is that one reaction we all seem to strive Mulholland) allow the child to the total care of our children has for. Be it throwing a cookie sheet “stand up” supported, freeing the many dimensions, and that we do on the floor to make a sound or arms for movement. A seat low to not have to continually meet needs faking a loud sneeze, or anything the ground with wheels allows the that others perceive as important.

34 G LOSSARY

Definition of Terms:

ABDUCT: EXTENSION: NYSTAGMUS: To draw away from A movement which Involuntary, cyclical Abbreviations Special Equipment the middle of the body. brings the members of a limb into or movement of the eyeball. toward a straight condition, for COMMON MEDICAL ABBREVIATIONS: ANOXIA: Deficiency of oxygen. example, straightening out of the PASSIVE EXERCISE: Muscular arm; opposite of flexion. exercise without any effort on the q every Tumble Form Carrie Seat and ANTIHISTAMINE: part of the patient. qd every day Carrie Rover A medication FISSURE: used to treat allergies. An ulcer or qod every other day cracklike sore. PNEUMONIA: Mulholland Travel Chair Inflammation of the q wk every week ANTISPASMODIC: lungs caused primarily by bacteria, A medication FLEXION: q4h every four hours (q6h, Snug Seat used to relieve spasms. The act of bending or viruses, and chemical irritants. condition of being bent in contrast q8h, etc.) Evenflo 7 Year Car Seat to extension. RECTAL PROLAPSE: ASTHMA: Difficulty breathing, and Protrusion of bid twice a day the rectal mucosa through the anus. Rifton Bath Seat wheezing, caused by spasm of the GASTROESOPHAGEAL REFLUX: tid three times a day bronchial tubes or by swelling of A qid four times a day their mucous membranes. return or backward flow of SCOLIOSIS: Curvature of the spine. Columbia Bath Seat stomach acids or contents up into c with the esophagus. Mulholland Prone Stander BOLUS: A mass of food given SEIZURE: A sudden attack of invol- s without at once. GASTROCOLIC REFLEX: untary muscular contractions and p after Corner Seat Peristaltic relaxations. wave in the colon induced by the ac before meals BRONCHOSPASM: Graco Courier Soft Baby Carrier Spasmodic entrance of food; this will often SUBLUXATION: pc after meals narrowing of the bronchial tubes. produce to urge to have a bowel A partial or incom- Mead Johnson - Cleft Palate movement. plete dislocation of a joint. pt per tube Nipple DISLOCATION: The displacement of gtts drops any part of a bone from its normal HEMORRHOID: WHEEZE: A whistling or sighing A mass of dilated prn as needed First Years Infa-Feeder position in a joint. veins in the anus, which can become sound resulting from narrowing of po by mouth inflammed, causing itching or pain. the respiratory passageway. ESOPHAGHITIS: Inflammation of P.T. physical threrapy the esophagus. HYPERTROPHY: An increase in size R.O.M. range of motion of a structure or organ which does not involve tumor formation.

35 36 About NTSAD

National Tay-Sachs & Allied Diseases Association

National Tay-Sachs and Allied Diseases Association was founded in 1956 by Tay-Sachs and the Hunter syndrome (MPS II) Additional Resources Allied Diseases Sanfilippo syndrome A,B,C,D a small group of concerned parents committed to the eradication of Tay-Sachs Alliance of Genetic Support Groups (MPS III A,B,C,D) 35 Wisconsin Circle, Suite 440 and related genetic disorders. These parents shared a common tragedy; each Tay-Sachs and the allied diseases Morquio syndrome (MPS IV) Chevy Chase, Maryland 20815 are rare, fatal genetic diseases that 1/800/336/GENE had a child affected with a rare, fatal genetic disease. Maroteaux-Lamy syndrome primary affect young children.* (MPS VI) Children’s Hospice International Over the past four decades, NTSAD has grown in size, scope and stature. These disorders are characterized 700 Princess Street, Lower Level Sly syndrome (MPS VII) NTSAD services now encompass 40 genetic diagnoses, including adults by a common biochemical defect: Alexandria, VA 22314-2265 Oligosaccharidoses 800/242-4453 affected by a rare Adult Onset form of Tay-Sachs disease. Fueled by a part- the inability of the body cells to Mannosidosis nership of dedicated volunteers, gifted professionals and a distinguished dispose of certain metabolic waste Council of Regional Networks products. The waste products grad- Fucosidosis for Genetic Services (CORN) Scientific Advisory Committee, NTSAD programs of Public and Professional Cornell University Medical School ually accumulate in the cells of the Aspartylglycosaminuria Education, International Laboratory Quality Control, Carrier Screening, 1300 York Ave., Genetics Box 53 affected children, causing a variety Mucolipidoses (ML) New York, New York 10021 Family Services, Advocacy and Research serve a diverse and international of debilitating symptoms and ulti- Sialidosis (ML I) 212/746/3475 constituency. mately death, usually by early I-cell disease (ML II) March of Dimes Birth Defects childhood. To date, a cure remains Foundation NTSAD’s Parent Peer Group, a Family Services program, links families Pseudo-Hurler to be found for Tay-Sachs and the 1275 Mamaroneck Avenue affected by Tay-Sachs and the allied diseases to a unique lifeline of informa- polydystrophy (ML III) White Plains, New York 10605 allied diseases: ML IV 914/428/7100 tion, resource and support. The Parent Peer Group is coordinated by Glycosphingolipidoses Adrenoleukodystrophies National Hospice Organization volunteers who are themselves parents of affected children. A newsletter, Landing’s disease A-beta-lipoproteinemia 1901 North Moore Street, Suite 901 family directory and national telephone network, all funded through NTSAD, (GM 1 gangliosidosis) Arlington, VA 22209 Batten’s disease connects hundreds of Parent Peer Group families. An extensive lending Tay-Sachs, Sandhoff disease 703/243-5900 (GM 2 gangliosidosis) Canavan’s disease library is also available to families. Additionally, NTSAD sponsors an Annual National Institute of Neurological Fabry’s disease Cerebrotendinous Xanthromatosis Disorders & Stroke (NINDS) Conference and family scholarship program. (Trihexosylceramidosis) Cholesteryl ester storage disease NIH Building 31, Room 8A16 Bethesda, Maryland 20892 Today, NTSAD is recognized as a leading, non-profit, voluntary health organi- Gaucher’s disease (Glucosylceramidosis) Pelizaeus Merzbacher disease 301/496/5751 zation. For further information on NTSAD, please call or write: Niemann-Pick disease Pompe’s disease National Organization of Rare (Sphingomyelinosis) Refsum’s disease Disorders (NORD) Metachromatic Leukodystrophy Tangier disease P.O. Box 8923 National Tay-Sachs and Allied (Sulfatidosis) New Fairfield, Connecticut 06812 Wolman’s syndrome 1/800/999/NORD Diseases Association, Inc. Krabbe disease (Galactosylceramidosis) 2001 Beacon Street, Suite 204 * The exception being Late Onset Tay-Sachs disease: Farber’s disease a rare disorder affecting adults that is commonly Brookline, Massachusetts 02146 misdiagnosed as multiple sclerosis or similar (Lipogranulomatosis) muscular / neurological disorders. Additionally, (617) 277-4463 symptoms of manic-depressive psychosis are Mucopolysaccharidoses (MPS) present in nearly 40% of affected persons. A newly (617) 277-0134 FAX recognized disorder, the course of Late Onset Hurler syndrome (MPS I-H) Tay-Sachs disease is not completely known. It appears however, that life expectancy is probably Scheie syndrome (MPS I-S) not reduced.

37 38 National Tay-Sachs & Allied Diseases Association, Inc. 2001 Beacon Street Brookline, MA 02146 (617) 277-4463