These are the instructions I give to anyone spending a short amount time with Crystal!
1. Diet: Supplements, Tolerex, amino 75, juice+ ,calcium, Vitamin D, Cornstarch, Prunes, Juicy Juice, multivitamin. *All of these items are mixed in with her juicy juice on a prepared set schedule with some being alternated at different feedings during the day.
2. Food allergies: Milk All dairy (cheese, butter, nonfat milk, powdered milk, cream, whey, caseinate, sodium caseinate,…) Meat All items that are a product of meat (for example: eggs) *all food items that are prepared with any of these items in it
3. Acceptable foods: plain cereals (served dry or with breast milk) Cheerios, Kix, Corn Chex, King Vitamin,… plain crackers, plain chips, plain popcorn, plain fruits (table food or baby food), plain vegetables (table food or baby food), Plain pretzels, Spaghetti (with no meat or cheese) Plain noodles.
4. Drinks: No milk! I prefer: Juice, water or breast milk She can have anything else: Kool-aid Pop
5. The reason: With Spinal Muscular Atrophy, the source of the problem is found in Chromosome #5. It is the SMN1 protein gene. For some reason, this causes problems with metabolizing and digesting complete proteins. These are primarily found in meat and dairy. Those found in vegetables and grain and such are not complete proteins and do not seem to cause a problem. Some of the side effects from eating or drinking milk or meat products are constipation and extra weakness and floppiness, along with additional problems with secretions. I have been told that soy protein can cause some weakness as well, but I have been experimenting with soy dogs and soy hamburger. Giving her small amounts a week and now we are up to as much as once a day and are still not seeing any ill effects from it.
Handling with care: 1. Picking her up-One hand supporting her head and neck. The other hand supporting her bottom and legs. 2. Diaper changing-DO NOT LIFT BY LEGS! Carefully roll her to one side, then wipe her clean, then gently roll her to the other side and wipe and clean. Keep in mind you will need to move her slow and place one hand behind her shoulder so her body will be evenly rolled. For real messy diapers you may lift her legs only to her trunk by the thighs and then place your hand under her tailbone to lift her bottom up. She likes playing peek-a-boo with her toes, it tends to get her mind off the discomfort. 3. Why the joints are so fragile-joints are not only held together by bone and cartilage, in fact, a lot of their security comes from muscles, tendons, and ligaments. Crystal shows that she maximizes at about 10% of the normal muscle mass, leaving her joints very weak and unstable. In addition to the muscle weakness, since she is not as active as a normal child she experiences many problems with loss of Range of Motion (ROM) in these joints and other areas of her body. Without the full use of ROM the body feels the flexibility is not needed so the tendons and ligaments that help hold the joints together as well tend to tighten and shorten, leaving the area stiff and unable to extend to its full range of motion. If sudden force is applied to some of her joints they may be dislocated and/or tendon and ligaments may be torn. 4. Never Ever Ever-Use any of her limbs to move her in any way. Lifting, Carrying, Sliding, Going from lying down to sitting…should all be done in a manner that protect all joints, even the simple little ones like her fingers, toes, neck…All joints should be protected, however her hip joints are especially fragile because of such limited weight bearing on her legs and hips, her ball and socket joint in the hip has not developed properly and is partially dislocated already. Please avoid extending either thigh in toward the other leg because this position turns the ball away from the socket making it easy for injury to occur. 5. Crystal's pet peeve-Crystal tolerates her limitations very well, but the one thing that will make her attitude unbearable and make her lose interest in working with any care giver is being put into positions that are simply beyond her abilities. Big example would be her head control. Upright she maintains well control, but too much of an angle forward or backward is too hard for her to hold her head up. Especially forward because her face would be facing down. In time you will get to know her and her limits. For a while you may feel more comfortable being over protective of her positioning, but in time it is my desire that you will learn to let her do as much as she can. For example if her head is up you would not need to support it, and even leaning forward up to maybe a 25 degree angle she can still hold it. It is good for her to use the muscles available as often as she can, so in this case you would not begin supporting her head until it is beyond the 25 degree angle. The less she uses her muscles, the faster they will shrivel, atrophy and weaken.
Things you can do to help: These therapies are not expected of you. It is only my desire to teach you of ways that can further benefit her throughout her day, but only if you have time and you feel comfortable following through with such tasks. 1. Range of Motion Fingers Wrists-patty cake patty cake is a fun way to disguise this therapy Knees (bending and straightening)-she loves the floor slide for this Lower back-again the floor slide Ankles-is easier with the knees bent. Leaning forward in a standing position works well. (Never stand her without her braces on.) 2.Traction-stretching her scoliosis curves. A. Stretching the thoracic region. Have someone hold her pelvic area while you hold her sides along the upper rib cage. Make sure that your help is holding tight enough so that the pelvic and below do not move at all. Slowly pull her toward you allowing her spinal vertebrae to stretch away from each other some. Then begin bending her upper body from left to right stretching the upper half of the spinal cord. Again slowly in a stretching type motion. B. Stretching the lumbar region. Have your helper at this point begin pulling the body toward her again attempting to stretch the spinal vertebrae away from each other some. Then begin moving the lower region from left to right in attempt to stretch the lower potion of the spinal cord. C. Correcting lower back hyperextension. After stretching the lower spinal, while the spinal vertebrae is still extended lift her knees toward her chest and hold a minute. Then let her relax and repeat as many more times as you and her can tolerate. 3. Standing-Crystal just loves standing and playing like the other children, but gets to do it so seldom because she needs one on one attention at this point to protect her from any falls. But with her braces on she would love it if you would. She does fatigue easily, so you wouldn't want to do it for any longer than 15 minutes at a time and at least give a 30 minute break between each standing session. She loves to stand at the sink and wash her hands also. Never Ever allow her to bear any weight on her feet and ankles without her braces on. When putting her braces on be sure to get all the wrinkles out of her socks and straighten out her crooked toe. These can cause Crystal discomfort making her standing not as successful. 4. Rolling-This is an especially good exercise for Crystal because it reaches so many different muscles all in one activity. She can easily get stuck and you will soon learn how much she hates that, but if you establish trust with her and always be there for her, while rolling then you will be successful at playing with her on the floor. I sometimes just have to get plain silly so she will have fun doing this. Sometimes all of her brothers and myself roll with her. Hey, whatever works.
Weather tolerance: 1.) 40 degrees and below-stay inside and cover nose and mouth for travel and emergencies 2.) 40 to 55 degrees-stay inside, but do not need to cover mouth and nose for travel and emergencies 3.) 55 to 80 degrees-May go outside with no limitations 4.) 80 to 90 degrees-May go outside for a period no longer than 15 minutes an hour 5.) 90 degrees and above-keep inside *Reasons: Breathing in the cold air causes Crystal to produce excessive secretions that turn to mucous and stay on her voice box since she is unable to cough hard enough to kick the mucous up and over into the esophagus or spit it out. Being exposed to hot weather exhausts her and fatigues her. She basically will become even more weak and unable to accomplish even the simple tasks for her.
Just some things I think about: