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Raising a Child With a Neuromuscular Disorder:
A Guide for Parents, Grandparents, Friends, and Professionals
by Charlotte E. Thompson, M.D.
Oxford University Press, New York, NY
Publisher's Book Listing - Includes jacket notes and author profile
[Note: I managed to find the author's email address, and we exchanged email about this review. I have added some comments structured like this one based on that exchange.]
As the parent of a child with JDMS, and keeping in mind that most people reading this review will be likewise, I've slanted my review to look at this book through our eyes. Much of the book deals with various forms of muscular dystrophy, and JDMS gets only scant treatment. Also, our family has been fortunate enough to not have to deal with severe disability; I will not be able to give anything more than general comments in this area. Still, most of the book is useful and practical.
The introduction to this book strongly suggests that it not be read cover-to-cover, as that could be overwhelming. Instead, the author suggests using the book as a reference to find advice on the specific topics covered. When read serially, as I did, many chapters cover some of the same ground, but when read as suggested, each chapter really does stand alone pretty well. With one major exception, outlined below, I agree with this strategy, and I will likewise review the chapters one at a time. Feel free to read my chapter reviews all at once.
Most people who read this book will find it only if they're already in the situation where they need it; that is, few, if any, people will seek out this book unless they have or know a child with a neuromuscular disorder. Assuming that audience, I recommend that several of the chapters should be read immediately, as they provide general information that may be quite valuable, especially if you have only recently received a diagnosis. I suggest reading chapters One through Six without waiting to use them as a reference. While that may seem daunting (over 90 pages), more than half of those pages are descriptions of dozens of different neuromuscular disorders; most people will only be interested in just one, the one their child has (probably JDMS), and the rest can be skimmed. These chapters can be read in about an hour.
One notable area that this book is sorely lacking throughout is Internet friendliness. Mentions of the Internet are rare, and many of those seem skeptical and cursory, and seem to have been placed as an afterthought. There is not a single email address or web URL anywhere in this book that I can find, especially including the resource directory. I searched for, but was unable to find, an online companion to this book, which is an increasingly popular tool to make books more useful. There are no directions or links to help the reader find online information or support groups. For a book published in October of 1999, these are egregious failings, especially, for obvious reasons, to this reviewer.
[Dr. Thompson agreed that there should have been more and better Internet references. However, the book was 4 years in the making and they simply ran out of time and resources to implement this. She talked about adding such a resource in international and other future versions of the book. I offered to assist her is creating a web presence/companion for the book; I have not heard back on this yet.]
Another area that is given insufficient treatment is faith. The vast majority of people have some faith system on which they rely. Faith can be a crucial coping and support mechanism that is all but completely ignored in this book save for the final chapter. The majority of people that I've been in email contact with through the diary have mentioned how their faith has helped them through it. Perhaps the author does not have a strong faith to rely on, but it is valuable, and should not have been so thoroughly ignored here.
[The author stated that she has a strong personal faith; perhaps I was incorrect to have called it into question. She went on to say that faith is something you have to be careful about discussing. I strongly disagree with this; this is nothing more that Political Correctness run amok. Families of faith could draw strength from such a discussion, and other can safely ignore it.]
One: Coping With the Diagnosis
This chapter describes the process most people go through when they learn a child is diagnosed; the shock, numbness, denial, anger, and grief. There's a lot of good suggestions for handling these stages, and channeling your energy into something constructive. It talks about dealing with stress, and having creative and fun outlets for both parents and children.
There are some excellent suggestions and ideas in this chapter. Unfortunately, just about everyone reading it will have already gone through the coping process and moved on to other issues. I can see a lot of value for someone who is "stuck" at one stage of accepting a diagnosis, and may not even recognize it. In some ways, this was a difficult chapter to read because of how it brought back the experience of our own diagnosis.
Two: When to Ask for a Second Opinion
There are a lot of valuable ideas in this chapter, which talks about getting a correct diagnosis, and finding a primary medical caregiver who is both caring and knowledgable. We are warned against doctors who are arrogant or controlling, or who do not take the time necessary to properly diagnose a rare condition.
We are walked through the various tests that should be performed before a final diagnosis is made: clinical exam, blood tests, EMG, MRI, muscle biopsy, and so on. The author believes that a muscle biopsy is indicated for every child suspected of having a neuromuscular disorder, including JDMS. This is not necessarily so, as Julia never had a biopsy (she had an MRI instead), and still received a positive diagnosis based on clinical and lab findings.
[The doctor said that she, along with other experts, does not agree with the use of MRI instead of biopsy when making a diagnosis.]
The point of this chapter is that you should feel comfortable with the attitude and thoroughness of your doctors. If there is any doubt at all, a second opinion is encouraged, and most medical plans will pay for one. Seeking a specialist may be necessary if your regular doctor doesn't know what's going on.
Three: Neuromuscular Disorders: History, Incidence, and Genetics
This chapter briefly discusses the history of muscular dystrophy, then talks at length about the causes of these diseases through genetics. Although there is a more detailed discussion in an Appendix, the description here is adequate for most people. It talks about the basic concepts of inheritance and genes. It also mentions genetic counseling, which will likely not be needed for most JDMS patients.
Four: Neuromuscular Disorders: Descriptions and Treatment
Chapter 4 is the listing of the various neuromuscular diseases. Each disease lists a history, incidence (rate of occurrance), inhertiance, gene site (if known), diagnostic criteria, and disease treatment and complications.
Skipping directly to the Myositis section, under Inflammatory Myopathies, there is only sketchy information. I was very disappointed in this section. Given that this is a book about childhood diseases, the discussion of JDMS is all but non-existant. The incidence is listed as "unknown", when the incidence is known and the estimates are improving every year, and that African-Americans get it at a higher rate. The Sex says "unknown", when it is known that girls get JDMS at a higher rate than boys. There is talk of complications (like calcinosis), but there is no indication of how common or uncommon they are. One glaring error is a reference to "Groton's sign", which should be "Gottron's sign".
[The Gottron's Sign error was characterized as a typo. Everyone know I'm certainly not immune to typos, either!]
Inconsistencies and inaccuracies like this, in the one area that I am knowledgable about, make me wary about believing other, similar information. Perhaps the author ought to have done more research.
[Dr. Thompson said she has extensive personal experience with JDMS, even characterizing it as her favorite neuromuscular disease because it's treatable.]
Five: Your Medical Team
This chapter is devoted to selecting and nurturing a relationship with a team of medical professionals, including doctors, nurses, physical therapists, social workers, and especially secretaries. There are tips on how to find and pick these people, and some common-sense suggestions like maintaining a notebook, using a tape recorder, and keeping a record of all tests performed.
One important point that is made here is that parents are the best experts about their children. No one knows them better, and no can detect subtle changes in symptoms and behavior like a parent. Kids themselves are usually very in tune with their own bodies, too. Any professional who does not actively seek out input from both patients and their parents is not doing their job.
The chapter goes into discussion about the various medical specialties that may be necessary, including Occupational Therapists, Orthotists and Orthopedists. Finally, there is an excellent section on establishing a good relationship with your doctors, with helpful suggestions like being on time for appointments and with payments, using good phone etiquette, and an occasional thank-you note or small gift as a show of appreciation.
Six: Navigating the Medical Maze
We all have to deal with insurance companies. This chapter is an excellent look at this problem, the attitude you need to take when dealing with them, and some practical suggestions. The problem is that you and your insurance company are usually at odds - you want the best medical care and coverage possible, and they want to pay out as little as possible. Once this is understood, it becomes easier to take the steps necessary to get the benefits you are entitled to.
The recommendataion that runs throughout this chapter is to get outside help. The author recommends finding an advocate for your child who will act as a go-between for you. She also suggests talking to other parents in the same situation, which is not always practical, especially with a disease as rare as JDMS.
Other good ideas include maintaining a meticulous paper trail, not only of medically inportant information, but also of all insurance company documents. Doing this will prevent you from having to request copies of documents unnecessarily, a practice that can jeopardize the relationships you have spent so much time culitvating. They also suggest utilizing state services to help with costs and to advocate for you to insurance companies.
Seven: General Medical Care
When our kids are afflicted with a serious, chronic disease, it's very natural to focus all our time and energy on fighting that disease. However, this chapter in the book stresses the need to consider the rest of your child's health as well. Even though we're taking them to specialists and therapists frequently, we should still take them to see their regular pediatrician regularly to make sure that their general health is not being neglected, and that immunizations can be kept up to date, if possible.
Topics for discussion here include: proper nutrition that can prevent a host of problems, especially constipation and obesity; proper eye care, although no direct mention is made of eye problems that can be the result of drug (like Plaquenil) side effects; and proper dental care. Dealing with the pain that some procedures can cause is discussed, and more detail is covered about lung problems that can occur.
This chapter makes some important points about maintaining good general health. A lot of this advice makes good sense for everyone.
Eight: Special Needs
Often, neuromuscular diseases that children get are progressive; that is, there is no cure or effective treatment, and over time, they require more and more special care. JDMS patients don't usually progress this way, but there are times when special needs must be addressed, especially for rapid-onset JDMS kids. Fortunately, these times are usually temporary, so much of the advice here may not be needed.
The theme of this chapter is to make every step that you take a step towards making your child more independent. Every adaptation should make it easier for the child to take care of themself; the more they can do for themselves, the more self-esteem they will have, and the better they will feel about their situation. For example, buying clothes that are easy for them to get into and out of gives them one thing they can do on their own.
There are detailed discussions of personal hygene, equipment (like wheelchairs and scooters, walkers, and lifts) that can help a child gain independence, travel consideration, accessibility of homes and public places, and safety. The reader is strongly cautioned about the overpriced nature of practically all medical equipment, and is wisely urged to seek out used equipment, or even borrowed equipment, when possible. There is also a section on computer use that is very sketchy and general; while it does encourage computer use by kids, it give practically no useful directions to enable it to happen.
Nine: Tips for Mothers, Fathers, Grandparents, Brothers, and Sisters
There's a lot of very useful and practical advice in this chapter. In summary, families and caregivers should not put their lives entirely on hold to care for a child with a disease. The key advice here is for every individual to make time for themselves on a regular basis, and couples to make time for each other as a couple on a regular basis. Without that opportunity, people will grow resentful and, ultimately, depressed.
The key strategy to accomplish this is to establish and nurture a strong support system of people who are willing and able to help out. These can be extended family, friends, neighbors, or even hired help, visiting health care workers, and community programs. Good practical suggestions are made on where to look, how to ask, and what to do to keep them involved. Lots of good stuff here.
Special attention is given to certain roles in a child's life. Fathers and Mothers obviously have special responsibilities and pressures, and have to deal with feelings that no one else has. Grandparents come from a different generation and can have difficulty dealing with the situation, or may be a godsend. Siblings can feel neglected, pressured to help even if they don't want to, and left out of the loop. In each case, good communications are essential, and again, it's stressed that each person and couple be given time for themselves.
Ten: Tips on Coping With Your Child's Ups and Downs
Dealing with your child's feelings about their disease can be difficult. Many people, especially men, have difficulty acknowledging or identifying their feelings, never mind communicating and describing them. However, feelings are the key to this chapter, and to dealing with the roller coaster ride that very much defines dealing with a chronic disease. This is one of the most important concepts in the whole book.
Kids in this situation most want to be understood. They have anger and fear and frustration, but often don't know how to express them, so they act out. The answer is to find a way to understand your child's feelings. Listen to them, pay attention to their body language and other non-verbal communications, and let them know that you understand. If you can't do this, getting outside help may be a good idea; the book talks about counseling, art therapy, and support groups (mentioning online support only in passing).
The latter part of the chapter talks about ways to prevent problems. Realistic goal-setting, and focusing more on what the child can do as opposed to what they can't is important. Address a child's fear and anxiety by letting them take as much control as possible. Finally, quality of life is discussed, including having a good attitude, and trying to live one day at a time.
Eleven: Helping Develop Your Child's Coping Skills
The key concepts of this chapter are building self-esteem in your child, and working to make them as independent as possible. These concepts are reinforced by encouraging discipline in our kids. We need to be aware that children are very manipulative, and will do anything to get what they want, even play one parent against another. We need to be aware of this and enforce fairness whenever possible. Our kids really want to be treated as much like normal kids as possible; when we do this, is builds the self-esteem and independence that they need.
Practical suggestions include mealtime discipline, chores with a reward system, and acknowledging and celebrating triumphs. This is a very uplifting chapter with a lot of good ideas. Remember, when our kids are coping well, it's one more way that helps the rest of us cope.
Twelve: Finding the Best School Program
This is a very important chapter for school-aged children with muscle problems. It talks about how to help you and your child cope with the delicate balance between having special needs met and being as normal and accepted as possible.
The problem is that many school systems, especially public schools, are not well equipped to deal with children with special physical and educational needs. Their budgets are often minimal, and their staff may not be properly trained to handle it. This requires parents to be especially dilligent in dealing with school personnel, much as if they were dealing with an insurance company. This chapter discusses laws that cover this situation, and how to ensure that you are receiving all the benefits you are entitled to under them.
I learned a lot about the process of securing an education for a child with special needs. It can be a great deal of work, and requires a lot of attention and monitoring to make sure both that your child gets what has been agreed to, and that changes to the plan are made as necessary. If you have a child in this situation, this chapter may be able to teach you a lot. Fortunately, we haven't had any problems like this, and Julia's school has been very cooperative, but I feel a lot more confident that we could handle it if it ever came down to this.
Thirteen: How to Be a Strong Advocate for Your Child
This chapter summarizes the need to represent the needs of your child, and the ways to do it. It requires good organizational behavior, good communications skills, and a lot of persistence. It can be difficult, and the temptation to take the easy way out will happen often, but the benefits of strong advocacy are many.
Most of these ideas and concepts are covered in other chapters, but it's good to see them taken all together. The need for a support system is outlined, and ways to build one are described. You are also encouraged to find and use lawyers and other outside advocates when necessary.
Fourteen: What Lies Ahead?
It is always tempting to look ahead, sometimes far ahead, to predict or speculate about the future. We are urged here to live in the day and not beyond. To worry about what may or may not happen is unproductive, and usually is detrimental. We should channel these energies into coping and making a difference.
As parents, we're urged to keep our kids as active as possible. We should encourage a healthy daily routine that expects them to do as much for themselves as possible. This chapter is brief, but has an important message that should not be taken lightly.
Fifteen: Questions and Answers
This chapter is a collection of questions that the author wants to address, but didn't seem to fit in anywhere else in the book. There is no theme here, just Q & A. Many of the questions are very specific to certain problems and conditions. I recommend reading each question, and only read the answer if it seemed relevant.
Sixteen: Surviving the Loss of a Child
This final chapter is one that would be difficult for any parent to read, and I was no exception. However, in some cases, this topic needs to be addressed. Fortunately, this situation is relatively rare for JDMS patients.
The advice in this chapter make sense for anyone who has lost a loved one, but the loss of a child is every parent's worst nightmare, and requires special attention. No parent wants to think about it, but this section forces us to, even if only briefly. There are a lot of suggestions for coping with such a loss here; where to turn for help and support (family, friends, and counselors), how to deal with it on a day-to-day basis (allowing time for oneself; grieving), and rejoining the world in due time.
Faith is briefly mentioned in this chapter, the only mention in the book, but not enough in my opinion. The loss of a child is a watershed event in the life of any caring parent, and faith can be the difference in acheiving acceptance or at least adjustment. We are obviously a family of faith, and that faith has made a tremendous difference for us. I really wish it had been covered better in the book, and especially in this chapter.
Appendices and Glossary
Appendix A: The ABCs of Genetics is a brief, non-technical discussion of genetics. It goes into somewhat more detail than in Chapter Three, but still keeps it simple. For people who don't already have a good grasp of genetics, this is a good start.
Appendix B: Tables is almost a rehash of Chapter 4. It's a tabular listing of virtually every known muscle disease that affects children along with some basic background information. The DM/PM listing will probably not teach you anything.
Appendix C: Chores Chart for Children is a one-page listing of age-appropriate household chores children can be expected to perform. The list is very basic, and will need modification depending on individual perference, and on what your child is capable of.
Appendix D: Resource Directories is a listing of agencies that can give you information. It's listed by disease, then state-by-state and country-by-country. This section is sorely lacking. Off the top of my head, for JDMS, there is no listing for the MAA, or the AARDA. The MDA is listed, but there's no indication here that there's anything there for JDMS patients (there is). Again, there are no Internet listings whatsoever, rendering this section all but worthless to those of us who have come to rely on the Internet for information. There should also be listings for major medical centers that support and treat these diseases (probably in the state-by-state section).
Glossary contains, as you would expect, a list of terms with their definitions.
Suggested Readings has a list of good references, but again is not very comprehensive or up to date.
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