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Dear parents and caregivers. It is imperative that you have support while caring for your child. SMA is classified as a Terminal Genetic Disorder and make no mistake about it it takes knowledge and effort to care for these kids. There is strength and knowledge in numbers and therefore I beg that you join http://www.smasupport.com/ chat group. The SMA Support chat group literally saved our child's life by supplying us with life saving equipment and information before our medical system would or had. There are over 140 families with mostly SMA Type 1 children on the chat though there are now Type
2's and 3's as well. The reality is is that kids following Dr. John Bach's protocol live longer and the proof is in the families you can talk to. FSMA has also adopted the NIV Protocol as well http://www.fsma.org/ I can not say enough about Laura Stants who started SMA Support Inc. and has lost 2 children to this terrible disorder. Please join the chat group today. I would just like to add that this web site was created so that you don't have to research while dealing with your child's devastating diagnosis. I was told by a few doctors not to reach out to parents that are
"obsessed with their child's care" that people on chat groups are "obsessed." Most Dr.'s have none to little of no experience or understanding on how to care for SMA patients on a daily basis. You have to drive the ship and this can only be done by negotiating with real, expressly written real information in hand. Please download as much as you need from this site so that you can better advocate (fight on one's behalf) for your child. Caring for children with Spinal Muscular Atrophy is one of the most joyful experiences. There is a parable in Judaism that says, "One who preserves one soul is considered as if he had
preserved a whole world." Unfortunately Dr.'s own beliefe systems come into play when action needs to be taken and plans created to look after our children (http://www.jeromegroopman.com/ "How Doctors Think"). Care should be determined by those that need it, not by those that supply or give it. Time and time again we in the SMA community hear of families struggling to make their doctors better understand that there are care protocols available that give families and the patients enhanced quality of life(watch this video on how to better communicate with your doctor http://youtube.com/watch?v=j3XxS-p31qY ). Though there is no cure for this terminal genetic disorder there are choices out there for families. The 3 choices I know of are: 1) Palliative Care
2) Non Invasive Protocol of Dr. Bach
3) Tracheostomy
Many of us have been told to "take our children home and love them until they die" and at first we thought this was our only choice; but it is not. We have choices and it is my hope that if you choose to keep your child around by treating them with quality of life enhancing protocols that you will have the tools you need
to advocate to the medical community in your area. Please work closely with the medical profession and advocate utilizing published written materials so that you can effectively argue why you want to care for your child (See my article on Advocacy under IN The NEWS on this site). If you are a newly diagnosed family please read the consensus statement and the SMA Family guide first. These two documents will give you a comprehensive background in a shortened format and bring to light what you are up against. After you have read the two documents please read my daughter's protocol and you can get a birds eye view into our lives. If you have any question feel free to contact
Brad or Maxine at asonginthisworld@gmail.com Click below to view Shira Fisher's Daily Care Protocol Shira Fisher Protocol April 16 2008.pdf Consensus Statement for Standard of Care in Spinal Muscular Atrophy Journal Of Child Neurology Standard Of Treatment And Care Of SMA Patients.pdf SMA Family Guide For The Treatment And Care Of SMA Patients FINALFAMILYGUIDE.pdf ADVANCED DIRECTIVE An advanced directive is a piece of paper that gives emergency services personal direction on what to do should your child loose their vital signs through cardiac arrest, pulmonary failure etc. This piece of paper tells EMS when to resusitate and when not to. This is a very important topic to discuss with your doctor. As Dr. John Bach states in his book, "Advanced directives
are valid only if the patient is fully informed about treatment options and in particular, the likely clinical course if they are not used." So, buy Dr. Bach's book "Management of Patients With Neurological Disorders! I know that in the begining when Shira was first diagnosed we were totally against tracheostomy but as time has gone on our whole conception of Quality of Life and what Life is has changed and so has our Advanced Directive or DNR (Do Not Resustate Order). It is important to speak with a physician that has a lot of experience with NMD (neuro muscular Disease) especially SMA. Some Dr.'s you might want to contact are listed on the Links page of this web site.
ADVOCACY The great humanitarian and especially childs rights activist June Callwood (http://en.wikipedia.org/wiki/June_Callwood ) said, "Help should be defined by those that need it, not by those that deliver it." Unfortunately you may find yourself dealing with a medical establishment that does not have the same beliefe system as you and will refuse to deliver the kind of care you want for your child ("How Doctors Think" http://www.jeromegroopman.com/ here is video of Dr. Groopman discussing his book http://youtube.com/watch?v=j3XxS-p31qY )though Unfortuanately most Pediatric doctors are missionaries of medical conventional wisdom. Most doctors are not willing to try unconventional treatments and are afraid to let parents care for their children using medical devices. Our children SMA Type 1 children are very weak and need help not conventional mdeity wisdom. As Sigmund Freud said, "People who want to make a living from the treatment of nervous patients must clearly be able to do something
to help them?" Most Dr.'s regurgitate information, they are not scientists they are skilled professionals that mostly can not think outside the box of education they have climbed into. Doctors mostly prefer to do what is acceptable over what is right. It is up to you as parents to fight like hell to find the right type of treatment for your child, back it up with logical written information and deliver it to your health care provider. Doctors constantly remind us that parents are their childs best advocates (fighters). So I say fight for what you believe in, what you want and think is best for your child. Ask your doctor for information in writing not just their biased feelings
about the subject at hand. I will now repeat myself and say, "Under these circumstances you will have to ADVOCATE (fight) for your child. Everyone in the medical system will tell you that parents are the best advocates for their children and they will hate you for advocating. Advocacy is a double edge sword. I suggest advocating in writing and leaving paper trails. For more information on how to more effectively advocate for your child please read my article on this site under In The News or you can use the self advocacy tool right here as a pdf Self_advocacy_ tool_e.pdf Know that you have 3
choices in caring for a child with SMA Type 1: 1) Strictly Palliative 2) Non Invasive Protocol of Dr. John Bach of the University of Medicine and Dentistry of New Jersey 3) Tracheostomy The choice is up to you as a parent. Don't believe anyone who tells you that your child will have no life. I know of many childrens dozens that enjoy their lives, are loved, cared for and give back affection to their syblings, parents and extended family members. Most Doctors know nothing about how to care for an SMA child. It is not easy but there is great joy and love in doing so. A
Doctors place is to support a family and make suggestions no dictate what should be done based on their own biased feelings, belief systems, lack of education, massive ego's, none to little experience with SMA, and more importantly no knowledge of your child/family dynamics and your beliefe systems. ADVOCATE WITH ALL YOUR MIGHT! ITS YOUR CHILD!!! BOOKS MEDICAL Management of Patients with Neuromuscular Disease
By John R. Bach, MD http://www.us.elsevierhealth.com/product.jsp?isbn=1560536047 Noninvasive Mechanical Ventilation
By John R. Bach, MD http://www.us.elsevierhealth.com/product.jsp?isbn=1560535490 PSYCHOLOGY The Suprising Purpose of Anger - Beyond Anger Management: Fiinding the Gift Marshall B. Rosenberg PH.D. Parenting Through Crisis - Helping Kids In Times Of Loss, Grief, And Change:
Barbara Coloroso What Children Need When They Grieve-The Four Essentials routine, Love, Honesty and Security: Julia wilcox Rathkey How Doctors Think: Dr. Jerome Groopman MD The Anatomy Of Hope -How People Prevail In The Face Of Illness: Dr. Jerome Groopman MD Close to The Bone:Life threatening illness and the search for meaning: Jean Shinoda Bolen,MD QUALITY OF LIFE BOOKS Quality of Life in Health Promotion and Rehabilitation: Conceptual Approaches, Issues, and Applications. (1996).
Eds. Rebecca Renwick, Ivan Brown, & Mark Nagler. Thousand Oaks, CA: Sage Publications. Quality of Life Research: A Critical Introduction. (2003). Mark Rapley. Thousand Oaks, CA: Sage Publications Still Me: Christopher Reeve CLINICAL TRIALS The best place to look for clinical trials for SMA Treatments is the National Institute of Health. I suggest going to their web site at http://www.nih.gov/ and entering Spinal Muscular Atrophy Clinical Trials in the search
box. Other resources are below. National Health Institute
http://www.nih.gov/
http://www.fightsma.org/
http://www.projectcuresma.org/
http://www.fsma.org/ http://medicine.utah.edu/neurology/research/swoboda/sma/index.htm
CARNIVAL Type I: Valproic Acid and Carnitine in Infants With Spinal Muscular Atrophy (SMA) Type I
Canada, Quebec
Hospital Sainte-Justine Recruiting
Montreal, Quebec, Canada, H3T 1C5
Contact: Monique Emond, PT, MSc 514-374-1710 ext 8278 montreal@projectcuresma.org
Contact: Sandra Reyna, M.D. 801-581-3551 sandra.reyna@genetics.utah.edu
Principal Investigator: Guy D'Anjou, M.D. COUGH ASSIST Cough augmentation with mechanical insuf ation/exsuf ation in patients with neuromuscular weakness Cough Assist.pdf DENTAL HYGIEN Tube Fed and Dentistry Tube fed and dentistry.pdf Calculus in children with G-Tubes Calculus in Children with G-Tubes.pdf Collis Curved Toothbrush http://www.colliscurve.com/ DIET SMA Nutrition from Dr. Bach's book, "Management of Patients With Neurological Disorders." SMA Nutrition by Dr. John Bach, Dr. Irving Haber, Jill Gaydos BS.PDF Dr. Kelly from Johns Hopkins on Muscle Metabolism. The AA diet is based on Dr. Kelly's findings Diet - Muscle Metabolism in Spinal Muscular Atrophy and Richard I. Kelley, MD.pdf SMA Type 1 patients can have muscle mass as low as 5% of regular muscle mass. If dieticians use the traditional BMI index (Body Mass Index) to calculate your childs dietary needs your child will become obese and will be exposed to amino acid overload. It is suggested to use the ZMM or Zero Muscle Mass index or to calculate your childs dietary needs by adjusting your calculations to take into account that your child
has upwards of 90% less muscle than a regular child. Nutritional Assessment of Patients.pdf Becaus our children will suffer from serious illnesses during their lifetime it is important to understand that our children suffer terribly when they go into the ketosis stage of fasting. Ketosis in laymen's terms is when the body goes into fasting mode i.e. before surgery or because doctors are too scared to feed our children for fear of aspiration. Ketosis then leads to affecting our kids fatty acid metabolic disorder. Our
children can loose a huge percentage of their movement through ketosis and fasting should be avoided at all costs even if it mains inserting a main line and using TPN or PPN. Nutritional_Acute Illness_Guidelines.pdf Dr. Schroth's Nutritional Guidelines. The below guidelines are just what they are guidelines. Most of the SMA parents I know do not aim to keep their children within the low 10% of the BMI index. I use a combination of information to calculate my childs dietary needs and use my powers of observation to monitor
her health weight wise. It is important that our children are not overweight as well as severely underweight. It is important that our children make gains but we must not forget that our children have a terminal genetic disorder that grossly inhibits the production of muscle. So, don't for a minute try and fit your child into the traditional BMI mold as our kids aren't regular children when we are talking about metabolism and physical make up. SMA General Nutrition Guidelines Dr. Schroth.pdf The full
article on the Amino Acid Diet The Full Article on the Amino Acid Diet.pdf Dr. Bach info regarding Heart and Carnitine Issues. Carnitine is more of an issue with many kids on the VPA studies but you can easily be tested. Diet - John R Bach, MD 2Heart Issues and Carnitine.pdf If you are thinking of putting your child on the AA diet please download the LMN and
edit the letter to suit your child. LMN for AA diet.pdf HOW TO CALCULATE YOUR CHILD'S DIETARY NEEDS CALCULATING YOUR CHILDS DIETARY NEEDS.pdf MECHANICAL VENTILATION Home Mechanical
Ventilation for Neuromuscular Ventilatory Failure Conventional Approaches and Their Outcomes.PDF NON INVASIVE VENTILATION (NIV PROTOCOL) Non invasive ventilation in laymen's terms basically means not cutting into the body i.e. Tracheostomy. This site is not against Tracheostomy but it is against the use of Tracheostomy unnecessarily. Tracheostomy is the old conventional wisdom when it comes to treating patients with NMD. There are definately stages of NMD as well as the patient's and families own personal wishes that will determine the type of ventilation.
The problem here is is that many patients and families are not given the choice because Past Conventional Wisdom has always dictated Tracheostomy. Below is a link to Dr. Bach's book and I highly recommend getting it so that you can use it as a reference. As time goes on you will become an old pro and what seems like a massive up hill learning curve will become common place in your day to day life. Preface in Doctor Bach's Book Non Invasive Mechanical Ventilation An estimated 16 million Americans have chronic obstructive pulmonary disease (COPD). In addition, 500,000 people in the U.S. A have
neuromuscular diseases, more than 500,000 have spinal cord injury or post-polio respiratory impairment, and 3-5% of the population have thoracic wall restrictive lung disease, sleep-disordered breathing, or obesity hypoventilation. In this era of cost containment, medical interventions are expected to be both efficacious and cost-effective. Both survival and quality of life are considered in equations designed to justify the cost-benefit for prospective interventions. Interestingly, non-invasive respiratory interventions are consistently preferred by patients over invasive alternatives. Use of these interventions has been shown to prolong survival while decreasing the
risk of pulmonary complications, need for hospitalizations and intensive care, and cost. Likewise, the use of respiratory muscle aids can optimize function, quality of life, and life satisfaction. Despite these facts, physician surveys indicate that only a small minority of patients who could benefit from these methods are ever offered them. This book is unique in presenting the various inspiratory and expiratory muscle aids and the outcomes of their use in patients with respiratory muscle dysfunction. -John R. Bach, M.D. Noninvasive Mechanical Ventilation
By John R. Bach, MD http://www.us.elsevierhealth.com/product.jsp?isbn=1560535490 QUALITY OF LIFE AND REHABILITATION
"The phrase is apt to cause disquiet. There have been those among us who have arrogantly judged, from a vantage point of power, the value of a human life. They have made decisions based on their assessment of a person's quality of life about providing supports to sustain that life. This attitude peaked in Nazi Germany, where such decisions were used as the basis for genocide. We like to think that we
have moved well beyond this perspective, but important decisions about people's lives are still being made from positions of power. Such practice is difficult to combat, especially in a period when responsibility of government in the area of human and environmental services is being cut back."- Introduction by J. David Baker (Quality of Life in Health Promotion and Rehabilitation) RESOURCES On the 3 sites below you will find links to all of your needs from Occupational Therapy device suppliers to Physical Therapy and Diet Informtaion. I use these sites
extensively. If you have any questions at all feel free to contact us at: asonginthisworld@gmail.com http://www.smasupport.com/ http://www.fsma.org.com/ SURVIVAL RATES There have been no
formal studies done on survival rates of The NIV Protocol and Tracheostomy and Type 1 SMA Patients but the unofficial statistics are 5% survival rate past 2 without trache or NIV or 95% survival rate with either. Dr. John Bach's books are clinical evidence in my world on how your child does not need to be trached to be cared for under most circumstances. There are always contradictions and each SMA Patient should be examine by a doctor that has experience implementing both NIV as well as tracheostomy. SMA Survival Rates.pdf |
