Neaker Girl

Friday, February 10, 2006
Little Man

Children are gifts from the heavens above and handed down to parents to care for, guide, love, and cherish throughout life. Expectant parents have many things to prepare for and to understand. Mostly, they have dreams and expectations that they want their children to fulfill as they grow. Some parents want their children to grow up and become teachers or firefighters. Most parents want their children to grow up loving themselves and believing in where their own abilities can take them.
You and your husband start preparing for the most wonderful and delightful event in your lives. A child is coming soon, and many things need to be done. You start picking out names for this child, and start painting or fixing up a bedroom for the child to grow up in. You start stocking up on the diapers, diaper wipes, and baby food. You can feel movement and begin to daydream about the coming months. How will this all happen? What will happen with your child? Will your child love you and your husband the way you both love this child now? What will this child look like? Will this child be a girl or a boy? There are many questions that you both have, and many things that you’re wondering about. The nearer the time comes, the more the anticipation and excitement starts to build for you and your husband.
At last, the time has come for this child to enter your world. There is much commotion, and everyone’s adrenaline is soaring. After 96 hours of labor, the doctor says just one more push and the baby will be here. You’re exhausted, but you still push with all your strength. All of a sudden, the child is here! Wow, the moment has finally come and everyone seems relieved that the birth is over, but there is a problem! You’re lost in the confusion that has just begun. You take notice to the baby, it’s a boy! This makes boy number four, but he is floppy and unresponsive to the entire world. There are doctors and nurses everywhere. The baby is not crying, and you and your husband are both trying desperately to get answers. The doctors and nurses whisk the child away in an incubator while working with everything they have to get this child to breathe.
In what seems to feel like a lifetime, but is only a few moments, the doctor returns to inform you that your child is ill, and they don’t know what is wrong. The doctor is speaking in terms that you can’t understand, but all you can really comprehend is the word “stable” at this time. Everything begins to become a huge blur to you and your husband; doctors and specialists are trying to explain what is happening. Your mind starts drifting into the past, the past that you don’t want to think about. It’s like you’re reliving the death of your third child all over again. How can this happen again?! The doctors told you that the first time was like lightning striking, and lightning never strikes the same place twice. What did you and your husband do to deserve this again? It has only been a year and a half since you buried your last child and you’re not done grieving yet! Your mind continues to drift off and ask many questions as you’re brought in to see the child in the intensive care unit. There is a delayed shock sneaking upon you and your husband as you both are unsuspecting to what your eyes are going to be seeing.
After scrubbing up and gowning up, you are brought to the baby’s bed side. You and your husband break down and begin sobbing uncontrollably while peering over the baby. Your child, your dream, your new life is hooked up to every kind of machine and instrument imaginable. This is your dream, your dream lying there, and it is now shattered. The baby is struggling for his life with tubes going into every part of his body. IV lines hooked in his little legs, patches hooked to his chest, and so many different alarms going off! What are you supposed to do, but hope for the best outcome.
Several months have now past, and you’re drained of energy. The baby has sustained more tests than can be counted. You and your husband have learned to adapt to the hospital life, going and coming everyday with the feeling that there may be no hope for this child to come home. The doctors finally have an answer, an answer to just one of the million questions that you have. There is a diagnosis. You are brought into a room and told by the doctor that your child has a rare disease called Myotubular Myopathy. There is very little known about this disease with the exception that these children don’t usually live past the age of one or two. You’re both told not to expect anything. You’re told that the child will never learn to talk, read, sit up, walk, and that the child will be like a vegetable.
At times, there is a child born into this world that is less than “normal” to your expectations, and you have no idea how to contemplate or handle this so called “gift.” This child has been born with a severe disability, a disability that was unexpected, unwanted, and not understandable. You feel as if there is no hope for your little boy because that is what the doctors and the specialists have told you. You live in a world of hopelessness, and no matter what way you turn, no one can give you any answers. Anytime you get your hopes up for something, you get nothing but a big disappointment. You’re exhausted from all the work in caring for your baby day in and day out. Hell, normal children are a lot of work when trying to meet their needs. Now you have to take on all the normal duties of childcare along with being a nurse, doctor, a therapist, and a magician all in one. Everything you do now is on a grueling schedule with demands unimaginable to everyone else. You live in a world by yourself, and also have fear in what is to come in time. You know that there will be a time when your child will no longer be with you, and that time will come before it should. You wake up every morning wondering if this will be the day that your child dies. Can you prolong the inevitable? Most people can’t, but the question never goes away.
Myotubular or Centronuclear Myopathy is the rarest form of 40 different types of Muscular Dystrophy, and is a congenital disease or illness that does not affect the bones or the blood, but affects all the muscles of the body. It is passed from an unaffected mother to a male child through the X chromosome, which is the sex determining chromosome. If the mother is a blind carrier, there is a 25% chance that she gives birth to an affected male child. If she has had one or more affected children, there is a 50% higher chance that the next child will be more severely affected. This disease can also be passed as a dominant or recessive trait, but the main passing is through the X chromosome. This disease mainly affects only male children with the exception of one or two female children. The reasoning for these girls that have been affected is that they have another rare condition that gives them an extra sex chromosome. This disease causes atrophy, weakness, and lack of muscle tone. For some unknown reason, along with the atrophy and weakness, this disease also causes an increased liver enzyme function and cardiomegaly.
There are five major physical characteristics of this disease. The first and mostly affected area is respiratory weakness and distress. This weakness leaves these children to have tracheostomies, dependent on ventilators to breathe for them, and open to constant lung infections. The next characteristic is their bodies remain floppy and lethargic, which leaves them wheelchair ridden and dependent on someone else to care for them throughout their entire lives. The third characteristic is their inability to swallow. This requires them to have a tube surgically placed in their stomachs for feedings. The inability to swallow also puts them at higher risk for aspiration of even their own secretions. The fourth characteristic is the limited eye movement, and the lack of blinking, causing the eyes to over water, and appear as if they are crying all the time. The fifth major characteristic is their elongated and thin faces with high palates, giving them ear and dental problems. Even though the actual prognosis or life expectancy is not yet known, medical technology has improved drastically, allowing these kids to survive longer and better lives in their own homes.
My youngest son , Forrest, has come to show us that he is growing up to become a little man and has outdone everyone’s expectations. He is a very smart and funny guy. Forrest was born in 1992 with many complications and serious medical problems of Myotubular Myopathy. It took nine months of extensive tests to diagnose his illness. He is totally ventilator dependent, gastrostomy tube fed, wheelchair bound, and totally dependent on someone for all his daily cares. This requires 24 hour nursing care in my home. He has the ability to speak around his trach and uses some sign language to communicate with people. He has learned many things in the last several years, but the best thing he has learned is that he can do anything he wants as long as he never gives up. Forrest attends school at Morgan Park Middle and is now in the sixth grade. He has been mainstreamed into a class with children that do not have disabilities, and the entire school has been very accepting and able to incorporate him into the agenda focusing on his abilities, not his disabilities. Forrest has brought much joy and understanding to our lives with all his smiles and kisses. As I have watched him grow and learn, he has come to show me that he is no longer a child, but beyond all the equipment, there is a little man that loves life and gives everything he can.
There many types of ethical issues when dealing with a child of this magnitude. The issues are on all sides of the street. As a parent, I have to make a decision as to what type of care or surgery I am going to allow for my son. A nurse needs to have an open mind when caring for a child with this type of high-tech, medical condition, and not dwell on their diagnosis and prognosis. People need to see the child behind all the equipment and big words and treat the child with respect, dignity, compassion, and love.
The nursing role is considered to be a very highly skilled position for the amount of care that has to be given. There has to be constant assessment of not only the child, but of all the mechanical equipment needed to sustain this child’s life. Nurses have to be able to adapt and be very clever with all that they do, because nothing is ever the same. The nurses become not only caregivers, but also teachers, problem solvers, mechanics, and therapists. They are required to be the eyes and ears for the physicians and specialists. They have to become part of the family and feel comfortable working in a home with the child. This puts a lot of stress and strain on them because there is really no direct supervision or direction for them. They have to work independently and as a team with the family all in one. It takes a very special person to be able to comply with all the needs of a child with Myotubular Myopathy.
Yes, as a parent, I’ve had to experience the ton of disappointments and shattered dreams with my son’s life. I have had to trudge through all the rig-a-ma-row and letdowns. I have experienced more hopelessness than most people will ever feel and feel like there is no end to the amount of things that I need to accomplish during the day. There are some days that I feel like I just can’t hang on anymore and want to give up! Raising my son has been one of the toughest things I have ever done, but I am still here doing it day in and day out. The best part to all this is what Forrest has brought to my entire family’s lives, nurse’s lives, and the community; he has brought us all hope, joy, acceptance, and unconditional love.
With these children born unto us unsuspecting parents, they are born unto the best parents there are. I will always be able to deal with the situation because God does not give us more than we can handle. These are special children that are given to special parents, and possibly are brought into this world to bring the world some awareness of a real happiness and satisfaction in life. The shattered dreams have faded away and become new dreams of encouragement and accomplishments for me and my family, because there are many more little men with Myotubular Myopathy out there needing care.

Posted by Neaker Girl :: 2/10/2006 09:54:00 AM :: 0 comments