In Home Care

Home Health Aide’s Guide:
Crystal Allbritton: Born on July 18^th of 1999.

I. Logbook: To assure that daily information concerning Crystal is communicated efficiently to each and everyone involved with her care, I have provided a log book for each of us to either learn from one another or pass on to the rest of us.  This logbook will be our most efficient form of communication, especially for times when it is not always possible for us to get together and have one-on-one conversations with each other.

To make logging questions, answers, or plain observations easier during your day, I will provide a micro cassette recorder for you to talk into as the question, concern, or comment arises through your day.  This way hopefully nothing will be missed and you will continue to learn.  At the end of the evening, I will type up what you have dictated and then respond to it if necessary.  I will print & post each days notes in a binder for you to access as you have time.  This will be a good reference tool for you to use if you need to refer back to it at anytime.

Since often issues may change, be added, or even be taken away, I will be posting in the logbook whenever I discover an issue that needs to be adjusted in her daily treatment plan. Another benefit of the logbook can be to share ideas with each other, especially from another one’s point of view other than Mom.  If you found a new way to get Crystal to accomplish a task with more ease or making it more enjoyable for Crystal or even if you have come up with a new play therapy, please share it with all of us.

*I expect everyone to keep up with the postings in the notebook.  You can read it while she is in her tub, stander, pooping, or anytime you can grab the book and read.  If you feel you are having trouble managing this, please let me know so I can relieve you while you are catching up on your reading.

II. Nutrition: I insist Crystal takes her amino formula with all of her supplements in it and I make sure that her fluid intake stays at a decent level.  Experts say with the amino acid diet, the supplements should never exceed to being more than 60 percent of their diet.  Getting her to eat & take her mixture is very tricky.  Currently, I am trying giving her one syringe of baby food at 9:30, 11:30, 3:30, 5:30, & 7:30.  This will give her two ½ jars for the day.  In addition, she should still sit with the rest of the family for their meal; it is not that she needs to eat more, but to be sociable with the family and if she desires table food, she can go ahead.  It is also good to stay in touch with her dislikes and likes so we can continue to offer table food that will appeal to her to give her self-prerogatives in eating.  I still give Crystal some of her favorite baby foods because that is an easy way to help keep her calorie count up.  She does grow, and she gains weight just maybe an ounce between each visit.  I even give Crystal a baby bottle with filtered water in it to go to bed with and she has been broke off the bottle since she was a year old.

Consumption of excessive dairy products: Although I will never accept Crystal ingesting straight dairy like a glass of milk or a slice of cheese, I do allow her to have a meal or product that has a rather high amount of dairy in it ONLY once a week. Meals that fall in this category would include such things as: macaroni and cheese, pizza (with cheese topping left on), cereal in a bowl with milk in it (even with most of the milk drained out as I have taught you to do), cheeseburger, bologna, and cheese sandwich, etc. If you are unsure if an item falls in this category, just ask.  Keep in mind, you need only record this meal if she actually eats more than ¼ of it, otherwise, you would have only offered it to her and it will not effect her body anyway.

Swallowing Issues And Eating:  Due to the voluntary muscles involved within the mouth and throat, and the excessive weakness adopted by her genetic born disability, Crystal has several issues involving swallowing while both eating and drinking all variety of textures.  It is the thinner liquids that show problems first by a simple disguised symptom such as choking, which Crystal has already displayed. For prevention of aspiration including silent aspiration, we carefully watch the amount within each mouthful. For example with her drinking, it is always best to use sippy cups without the spill proof rubber piece in it. This automatically limits how much she can take at a time, and she would not provide much sucking motion to intake much more than what the cup allows just being tipped. Since Crystal has already displayed problems with choking on thin liquids, the sippy cup is something we never steer away from.

When serving table food: Currently, Crystal’s swallow has gotten drastically weak.  We have had to resort to the above feeding plan because of this.  Frequency is always the best way to increase strength in her.  This is one reason we are insisting the baby food in the syringe it is quick & easy and allows her to continue practice the swallow.

A new issue Crystal is experiencing is the possibility of heaving GERD (gastroesophageal reflux disease).  It seems that when she gets too full, then she raises her chances of vomiting it all back up.  We suspect this to be acid reflux.  Therefore, what we are attempting to do is offer more frequent feedings at a smaller amount.  It is best to alternate food and mixture by serving the mixture on the hour and the food on the half hour.

Another way of continuing therapy for the swallow without filling the tummy is to offer her an occasional “mentos” or “StarBurst.”  This will increase er saliva increasing her need to swallow, but it does not fill her tummy.  These candies are also important to her to therapy her jaws which are also weakening and having tightening in their range of motion.

Because of Crystal’s swallowing issues, weakening in the mouth, and swallow reflex, Crystal will take a very long time to complete a meal. She often will get tired before she has really eaten anything. I have noticed that when she packs her jaws with food, she tires quicker disabling her from being able to continue. On occasions, it has been effective to hand feed her tiny bites, like the size of a pee. Another helpful tip would be to make sure her meals have had adequate time to cool down closer to room temperature. This will help trigger the swallow reflex.  It is also necessary to allow extended time for her to finish a meal. Serve her meals early, have them ready and sitting where she can see them and reach them. As soon as breakfast is over, go ahead and serve snack and let her eyeball it. If she nibbles a bite here and there, then that is fine too. Then an hour before lunch, remove the snack and serve lunch, and so on, which is pretty much the same thing that needs to be done with her liquids. Honestly, eating, drinking, and Tolerex fight against each other. Tolerex is obviously the number one priority, and we always make sure she gets what she needs of this. Liquids would be the next priority, but between the Tolerex and the liquids, Crystal could very easily get full and have no more room left for any eating and then vise-versa. So keep in mind to always have both food and liquid available and then try to keep a “happy” balance between them meeting Crystal’s nutritional guidelines.

Drinking: This is an exceptionally tough subject and one that has been experimented with for over a year now. Used to we would manually give her 2 ounces of water via a syringe each hour to keep her liquid intake up. Currently, Crystal does real well when manipulated with a cute cup, a baby’s baby bottle, the filtered water helped a bunch, and then I have had to settle with her drinking more Kool-aid than what I would like. Keeping the body hydrated is so very important for many reasons, but the most understand and easiest to explain is the circulation of blood. Within blood there is basically everything the body needs to live including oxygen, nutrients, minerals, leukocytes, red blood cells, and so on.  Nutrients in the blood include proteins, calcium, iron, and all that your body needs are transported through the blood.  The more water in ones blood system the faster and more efficient it will be circulating all of these necessities to its destination. This is especially important for oxygen, nutrients, and the leukocytes, which aid in fighting off infection. With all that Crystal has going against her physical well being, this is something within our control in which we can provide the best possible benefit for Crystal. I expect her to not only have a drink within her reach at all times, but a drink that she is highly likely to drink, served in a container that she is most likely to want to drink from. I also expect for her amounts by the ounces to be closely kept track of and marked accordingly on her Daily Chart.

Diet: Supplements: Tolerex, amino 75, juice+, calcium, Juicy Juice, glutamine, flax seed oil.  *All of these items are mixed in with her juicy juice and jello on a prepared set schedule.  It is recommended by the doctors and the dietician that she receive no more than 60% of her total ingestion of food and/or liquids to be consisted of supplements, as in the ratio is best not to go over 60%-supplements and then not less than 40%-of natural food or liquid sources.  Vitamin D is given to Crystal along with her morning dose.  These supplements are mixed together totaling 12 ounces.  2 ounces will be given when she wakes up and 1 ounce starting at 9, then 10, & 11.  At 12 noon she receives 1 ounce mixed with on teaspoon of cornstarch before she lays down for nap.  She will then begin her 1-ounce doses back up at 4 p.m. no matter what time she wakes.  Then 1 ounce at 5, 6, & 7.  Then wait one & a half hours til 8:30 and give her 2 ounces with 1 tablespoon of cornstarch. 

The cornstarch is added to the formula before sleep times to slow the metabolisation and digestive process to prevent any kind of fasting or ketosis through the long hours that Crystal endures without being able to receive any nutrition. Prunes are added when bowels are having trouble moving.

* Purpose of the frequency is to allow a continuous supply of amino acids to her body, while lowering the risk of toxicity with giving her too much at one time.

*Special note on the Tolerex formula: The formula cannot be given any sooner than 45 minutes from her last dosage.  The most effective way for the Tolerex formula to be applied to Crystal is through a consistent schedule. If you give it to her to soon after her last dosage then you will be presented with a problem of amino acid toxicity, which is very harmful to anyone, especially Crystal (a child of SMA).  It may cause her to vomit, increase her secretions to an uncontrollable amount, and it will make her “temporarily” much weaker than what is normal for her. Her symptoms can range from being mild, moderate to severe, depending on the level of toxicity. Mild symptoms would include a small cough, and falling while sitting more frequently, or not being able to reach as high, or even not holding her head up as well. Severe symptoms would include a severe cough and maybe even nasal drainage, having difficulty sitting entirely, and of course vomiting.  If too much amino acid is continuously given, then these symptoms will remain and simply appear to be a part of her disability.  Yes, she is weak, but toxicity makes her even weaker.  If you give her the formula too long after the last dosage, then you will be presented with the silent problem of fasting or ketosis (catabolizing your own muscles for energy). This problem is scary because its symptoms don’t show unless this is an ongoing problem. It is my goal with Crystal to spare absolutely all of her remaining muscle tone. As the protein is stored in the muscle, and she has so little muscle, she basically uses the protein as she ingests it and then very little is put away for storage. This is why the continual feeding throughout the day of the Tolerex formula, (not to overload, but make sure a supply is always there for her body to access.  If ever a dosage is missed, give it to her ASAP & then adjust the rest of the day’s doses accordingly.

·        Allergies: Antihistamines say on the label that they may cause drowsiness.  With Crystal, they cause drowsiness which shows up as weakness and in turn can ruin her whole day for therapy purposes. Needless to say, I do not allow her to take this type of medicine. Dairy (cheese, butter, nonfat milk, powdered milk, cream, whey, caseinate, sodium caseinate…) including eggs. Her system can only tolerate these substances in very small quantities. Never give her any straight dairy products like milk, cheese, ice cream or anything that has a high mixture of dairy in it. Meat can cause the same problems as milk but just not as severe and it may take a larger amount of meat to show symptoms. Generally, because her appetite is so poor, we don’t have to limit her intake of meat, but if circumstances were to change where she began eating more, then we would limit how much meat she is allowed to eat in a serving.

·        Always acceptable foods:  plain cereals (served dry) 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.

·        Drinks:  No cow’s milk!  Give rice milk instead.  I prefer: Juice, water… She can have anything else: Kool-aid, soda (diluted), anything you have to do to get her to drink.  You can even mix nestles strawberry or chocolate with her rice milk.  Bowels:  Counting the days from the last bowel movement is a must and I follow these steps accordingly: On the 4^th day of no bowel movements being reported I add a jar of prunes into the diet anytime throughout the day.  Then on the 5^th day, I wait to see if she will go on her own. If again no bowels are noted, then I apply 2 jars of prunes in the diet, each at a different sitting, on the 6^th day. The 7^th day of no bowels moving then, I would apply her medication, Myrolax, to water down her bowels. Any bowel movements that are small (especially after 3 or 4 days of not going) are not counted as an adequate bowel movement and we do not start the day counter over for them. I would ask, though, to still make a note for everyone to see. Until you learn to recognize what is considered an adequate amount for Crystal, please do not flush the toilet after a bowel movement so that I can guide you on being able to make the appropriate decision for her movements.

The reason: With Spinal Muscular Atrophy, the source of the problem is found in Chromosome #5 with the SMN gene. Running parallel to each other, the SMN1 gene and the SMN2 gene usually work side-to-side functioning as a team. It is the SMN1 protein gene, which is 95% of the time deleted from SMA children, but sometimes it still remains but is mutated and not functioning properly. The SMN2 gene can fulfill approximately 10% of SMN1 gene’s functions; however, this is by far not enough of a compensation for their bodies to be able to function normal. Not only does this cause a problem with the making and packaging of proteins that our body makes on its own; it also, for some reason, causes problems with metabolizing and digesting complete proteins that they ingest from their diet. 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 and so far have noted no ill effects with keeping the frequency and quantity down to a minimum. 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.

III. Handling with care:
1. Picking her up-One hand supporting her head and neck. The other hand supporting her bottom and legs.
2. Potty accidents and messes-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 big messes, you may lift her legs BY HER THIGHS only to her trunk and then place your hand under her tailbone to lift her bottom up to where you can reach underneath. 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.

6. Communication-treat Crystal as your friend.  Pay close attention to what she tells you.  Repeat it back to her to make sure you heard her right.  She often becomes instantly upset with a caregiver who misunderstands her and leads her to an activity she did not ask for.  She is a very reasonable little girl and most activities can be accomplished through compromise.

IV. Things to do:
1. Range of Motion Fingers, Wrists-patty cake patty cake and pulling the wrists back while washing her hands easy and sometimes-fun ways to disguise this therapy. Knees (bending and straightening)-Briefly, as you change activities with her, hold her in your lap facing you with her sitting on her knees allowing her to feel as much of the weight on her knees as she can tolerate, Ankles-is easier with the knees bent. Leaning forward in a standing position works well for the ankles. (Never stand her without her braces on.)

Range of motion is essential for all SMA children simply because, "If you don't use it, you lose it.”  Because our children are so weak, there are many muscles and joints that tense up that just are not utilized because our body’s triggers are not being used daily. For example, when you supinate and then pronate your wrist, that sends a message to our control center saying, "I need that motion." If we don't utilize this range of motion, then no trigger is sent; therefore, we believe we don't need it anymore and thus contractures are formed.

Contractures can be very painful, and can even further limit the abilities of your child. We as parents need to spend time throughout the day actively physically doing these different ranges of motions in the different areas for our children. I am not familiar with what is common with all SMA patients but I know what is common for my daughter. With Crystal, I stretch and rotate for her the following areas:
1.) We stretch the ankles joint and relax posterior tibial muscle. (Dorsiflexion)
2.) We pull the wrists from supination to pronation and then return and repeat.
3.) Shoulders: Abduct and adduct.
4.) Fingers: We flex and extend, along with relaxing the muscles.
5.) Lower back:  She is hyperextended so I stretch her back the opposite way from the hyperextension.
6.) Toes: Flex and Extend.
7.) Knees: Extend, straightening it out completely, then I flex going back to the posterior thigh until I feel tightness.
8.) Spine: From right to left, first securing the thoracic cavity, and then securing the lumbar cavity.
9.) This ROM is new so bare with me, I recently learned that Crystal was ulnarly deviated and along with bracing, traction and ROM we are hoping to correct this before it develops into a contracture. In simple terms: it is the motion that the hand would perform from left to right using the wrist joint as the point of flexion.
10.) Neck-lateral flexion: hold her shoulders straight and be funny to her ex. “what’s that behind you?”  “Can you see it?”  Have something behind her to the right & to the left as well.
11.) Shoulder-flexion: you will have to manually do this one, as I have not found a sneaky way to do it yet.  Arm must go straight out in front of her straight up until it stops, push only slightly beyond that point as she tolerates it.
12.) Shoulder-abduction: out to the side (same as above-do manually)

Included is a list of all the possible areas of the body that range of motion should be monitored frequently. Every Saturday from 10 am – 11 am, go through the checklist & do a spot check of Crystal’s abilities to see if any more areas need to be added to her daily plan.

Crystal’s ankle braces are very effective at keeping her ankles relaxed.  These should be kept on her at all times unless she is involved in an activity in which she cannot wear them i.e. the tub or rolling.

Her wrist & finger braces, however, must be used during sleep.  They accomplish two things at once: 1.) Keep her fingers in the neutral “relaxed” position 2.)  Keep her wrists in the neutral “relaxed” position not allowing ulnar deviation while being braced.  Wrists and fingers must be positioned and aligned properly before straps are applied.  While straps are being placed, be extra careful to make sure she does not wiggle out of position.  Straps should be snug but not so tight to leave marks or cut off circulation.

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. Do it for 10 repetitions for twice daily. 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 are 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.  Part of the problem causing the hyperextension (lordosis) of her lower back is having weak abdomen muscles.  Sit ups is an additional therapy to help this problem.

Stretching the spine: This is when you simply stretch her upper body down to the right while stretching the left vertebrae. Then you stretch her upper body down to the left while stretching the right vertebrae. This can easily be disguised in play with one-on-one with the caregiver leaning with her to one side and then to the next saying something funny like, “Woooh, we are falling!” Sometimes she gets in these playful moods when you can be silly with her like that. This is another range of motion technique to keep the spine limber and ready to be able to move as the body asks it to in all the different positions.

*Special note about scoliosis-Crystal began her scoliosis S-curve when she was 2 years old. She will end up having a spinal fusion to correct the severity of the curve at some point in her life. It is of the up most importance for all of us to work together all day, everyday, to keep her from putting any unnecessary hardship on her curve. I expect for all of us to make sure all day, everyday that Crystal is placed and KEPT in a sitting position that does not allow her to lean into her major curve which has developed to her right chest area. The reason why this is so exceptionally important is to hopefully spare Crystal from the need to have any more than one spinal fusion surgery done, where they will make an incision from the top of her spine all the way down to her tailbone deep enough to place rods in each and every disk of the spinal column. If Crystal develops a need for the spinal fusion before her spine has stopped growing, then as the spine continues to grow, the rods will cause her back to develop a crank shaft and another spinal surgery will need to be performed to place longer rods to adapt to her new length. Considering the seriousness of this surgery and the fact that it is most likely that any abilities Crystal has of her lower extremities will be then lost, it is one of my priorities with Crystal that no excuses will be excepted from her caregivers or even Crystal herself.

Stop the use of compensation-It is common for anyone with disabilities to discover easier ways to accomplish certain tasks for themselves, including children. Although this may be a good skill to acquire for some things necessary for them to survive; it also tends to progress their weakness because they are not utilizing all of their muscle functions. “If you don’t use it, you lose it!” is an understatement with SMA children. Bones, joints, tendons, ligaments, and muscles are all at risk for losing their functions at a rather quick pace if they are not only used but also used properly. End range is one form of compensation that Crystal is great at abusing because that is a comfortable resting state for her. For example, when you see her lift her head, don’t get too excited if her head is all the way back. She is not using any muscles to hold the head in that “end range” position. Another example of compensation is when she locks her elbow while pushing the joystick of her wheelchair. Because the elbow is locked, the effort requires no use of any muscles to push the joystick forward. Yet another example is while she is simply sitting and placing her hands on her legs to help her stay upright. Or if she is reaching up with the right hand, she will place her left hand down on her leg or a table to add other strength to the attempt to reach. If she is taking a break, while you are preparing her next activity or a drink etc., I don’t mind if she uses these techniques. But it is more productive for her therapy play if you do not allow her to compensate. You can put something in her hand to keep her hands busy, or gently push on the back of her head so it doesn’t go to “end-range”, or just gently bending her elbow to stop it from being locked is continually what you will need to do with her as you play and work with her. While she is sitting on the floor, she likes to bend her knees out in the frog-legged position because it helps her keep her balance. This is okay, but for constant periods, it can cause her knees to get tight, stiff, and then causing avoidable pain. It is also good to have her legs straightened because it does cause her to use other muscles to maintain stability while sitting. On some things, she already knows I don’t like her to do it and it may be as simple as looking at her with “that look” that she will learn to know as you say, “Crystal sit up straight.” or “Crystal bend lift your hand up.” Here is the list of things that need to be watched at and corrected ALL times:

·        Locked elbow

·        Lean on hands

·        Lean to the right imposing on her scoliosis curve

·        End range of head motion

·        Squatting in tub for long periods of time, more than 5 minutes and/or more than 40% of total time in tub

·        Her packing food in cheeks, taking too big of bites, etc.

·        Changing leg positions while sitting on the floor

Other items that need to be accurately observed and logged:

·        Coughing

·        Bowels

·        Gagging while eating or drinking (not choking) but like she is going to throw up

·        Signs of weakness

·        Signs of pain

·        Night sweats

·        Extra secretion reaction to dairy

·        Note anything out of the ordinary: Eating more, drinking more, less frequent urination, irritability, trying a new technique, participating in more therapy time than usual, participating less, etc.

·        Choking while eating or drinking

·        Range of Motion of all the areas specified within this packet

V. Mommy Words: I work with Crystal everyday, at scattered times all through out the day. I keep a schedule on what muscle areas we are reaching with each activity and I ALWAYS let her be my guide to what she can tolerate. 

I started out being brief with her sessions, and gave lots of rest times. Gradually I made the sessions last longer and longer increasing the times once a week or so, until she was at her maximum ability and tolerance level. I also grew in my expectations of her a little at a time. 

We do have testimonies when I was going to school full-time taking my first medical class and just didn't have time to work with her and still receive a good grade in the class. I watched during that 16-week period, Crystal get weaker and weaker by the week. She got floppier and fatigued easier as well. Then I had a month break in between semesters and slowly began approaching her with therapy. The good news is that she came back to life. It took almost the whole month, but she was able to go back to her originally abilities. Needless to say, I only attend school part time now.

The reason why I let her be my guide is that I figure that she is not going to push herself into fatigue. When she is tired she just stops. When she says she is done, then I accept that. I have seen her play real hard on some days, just overwhelmed with excitement that it even interfered with her naps. But the next couple of days she would suffer from fatigue. She would be weaker and floppy and even unbearably cranky. So I even put a limit to certain types of activities and I am religious about her sleeping schedule. 12-3pm is naptime and 8pm to 7am is bedtime. 

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. ROM of the ankles can even be put to play while she is standing by simply leaning her forward a little bit making her toes go closer to her shin. 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.

Scoot Walk:  This is the most amazing thing Crystal has ever been able to do. I never in my wildest dreams thought she would come close to doing this.  Crystal is able to stand unassisted holding onto such items as bars, your legs, your arms, your fingers, etc. and actually scoot her feet backwards, from side to side, twisting and turning, and then with all of her efforts, shifting all of her weight to the right side, she can take one normal size step forward unassisted as she holds onto something herself. She on one occasion shifted all of her weight to the left and advanced forward with her right foot. But all the other times it has been advancing forward with her left foot.  This is one of the abilities Crystal has lost with the breaking of her leg that I am hoping so very much to be able to regain in the future. This, along with all of the other weight bearing activities requires one-on-one supervision at all times not letting your hands more than an inch away from her side to be ready for that impossible to predict fall that is very likely to happen from time to time. It matters not to me if she goes two weeks without a near fall or not, I will not tolerate this activity and all other difficult activities of this nature being performed without your 100% attention AT ALL TIMES!

Free Walk:  This basically means that you as the caregiver are relieving Crystal of the majority of her weight, and assisting in the advancement of her legs forward by tilting the pelvic girdle forward somewhat to give her legs an additional boost. This is NOT a weight bearing activity. The purpose of this would be to stimulate the walking action for her legs, give her some pride in accomplishing getting from A to B, and to work head and trunk muscles. Be sure that her trunk is only being spot supported by your forefinger being just below the trunk and that she can lean to the right and left as she advances forward so that she will have the need to pull her trunk and head back up to the neutral position.

Assisted Walk:  Go Crystal Go!! This is the toughest of the 3 walking therapies. This is where Crystal is bearing almost all of her weight on her own.  She is receiving no pelvic or trunk assistance.  Basically, the only thing the caregiver does during this type of walking therapy is make sure she is aligned straight and correct, and that her upper body stays balanced and caught up to her lower body.  Now being that this activity is in fact the most difficult, her endurance is not very good at all. She may only be able to fully function at this for 20-25 steps or 5 minutes, but as she tires, she can still continue with you relieving a little of her weight for her and beginning a little pelvic assistance. Generally, her trunk is the stronger and may not need assistance for a longer period than the other two.  Keep going, the more tired she gets the more assistance you give.

*Special note of all walking therapies:  Especially after being down from a broken leg for 3 weeks, frequency is always the best way to be most effective with activities of such difficulty to Crystal, because this allows adequate resting time but yet also still offers her the opportunity to continue utilizing that function. But in addition to frequency, always remember that if you find Crystal not being able to participate in ANY activity including standing and walking, you can always compensate for her so she can then accomplish the task at hand. I am not saying by no means do it for her, in fact, I totally discourage this; I am only saying it is best for her to be able to still do the task with you providing assistance by relieving her of some of the weight.  There is no set level of assistance or weight relief to follow. It really will always be a judgment call per her ability level that moment.

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?

Floor Play-Be sure to allow adequate time throughout the day not just for rolling but also for just floor play because there the floor is where Crystal displays her most independence. She can play and experiment with her body and its parts to figure out different ways of moving them. If you leave her alone on the floor and peak in at her, you will catch her doing such things as: Scooting, moving her legs in various positions, lying down on her own, elbow weight bearing, and even rolling plus many more depending on what her thoughts provoke her to do.

VI. Ideas: All of the children and I have this little game that we play throughout the day. Whenever I am going to offer anything to them, I get all excited and say, "Who wants Kool-Aid?" or whatever, and they all raise their hands up high. And then the next time when they raise their hand I will say, "Uh oh, it is the other hands turn." to try to keep the arm strength balanced as much as possible.

Reaching: (for arms and shoulders)
Crystal really has a lot of toys that help work her arms and shoulders. She has actually defeated all of her goals for reaching. Her current goal is to be able to increase the height of reaching while standing.

Head control:  Swinging is probably the most enjoyable way to benefit Crystal’s head control. You will need to pull the swing up high while supporting the back of her head. As SOON as you let go of the swing it is a time race to get your other hand in place to support the front of her head before the swing begins coming back forward. If you fail to get there in time she will fall forward hitting her head and causing great strain to her neck. With both the front and back of the head supported as your hands stay with the movement of the swing, you and Crystal work together to get her head stable in the neutral position. When you think she has got it under control, ask her if she is ready, when she replies yes, proceed to let her go. Stay right by her ready to catch her head if her neck muscles give out, which happens quite frequently, so again no strain is caused on the neck. I am Crystal's slide. This technique applies fun to working hard to hold her head up. She absolutely loves it. She starts here and then slides down to a standing position.  Also, the easiest way to apply head control therapies into her day is simply by the way you hold her and walk with her. Allow opportunity for you to carry her upstairs or with you into the kitchen she can hold her head up high as you are walking. Set her on one arm to the right or left, you will want to take turns with each side, and then do not support her trunk or her head; just stay on guard for if she does go to fall. Move at a steady even pace, slowly and gradually increasing your speed. This is actually more of a work out than you would ever guess for her. It works both the head and the trunk. And the purpose of taking turns with the right and left arms would be to offer equal opportunity to both the right and left side of the trunk to develop its strength. By surprise, a windy day is also a very good way to work both the trunk and the head muscle areas. She actually really has to struggle to stay upright, will get tired, and then begin having some falls. 

Going potty: She stands at the potty while we pull her pants down, she goes potty, she stands at the potty while we put her pants back up, she side shifts (scoot walk) around to the side of the potty, she closes the lid, she flushes the potty and then we currently are trying to get her to side shift (scoot walk) back around to the front of the potty.

Elbow weight bearing: Best is on the floor or her elevated bed. Try reading to her, playing games with her, hide-and-go seek, be imaginative while she is lying down. She loves experimenting and moving her body, let her be free somewhat. Often she will maneuver in ways on her own that are good for her. This activity often coincides with rolling and will in turn work her head control as well.

Clean-up: Crystal's therapist all taught me how easy it was to get just a little more exercise for Crystal by helping her clean up her toys after an activity. I always hold the container away in different locations to cause her to have to reach using more trunk, head and arm control. The wonderful thing about this activity is that she actually enjoys cleaning up her toys.

VII. Equipment:
Stander: Primarily, she does her standing during breakfast, lunch and dinner. But the last couple of weeks she has really enjoyed the peace away from her little brother, Nicholas, while she is up high where he can't reach and has been asking to play with her little babies in her stander. We push standing because of bone and joint development. Once you stop and think exactly how much a normal two year old is up on their feet running around, you will see how little these SMA children are up. Especially the hip joint and the femur depend on weight bearing to be able to grow and develop properly. Even after being so aggressive with Crystal's standing and walking exercises, she still suffers from subluxation of both hips. Often Crystal will rebel against being placed in the stander and it is up to her caregivers to go that extra mile to change her mind. Some previous successes have been things like:

·        Having Crystal wheeled into the bathroom up to the sink and play in water. She can wash dishes or give her little babies a bath. She can play with blocks, bottles, cups, etc.

·        Since babies are her favorite, you can usually set her up with one of her baby bins and she will be occupied for a small period of time.

·        He especially likes her baby bin that includes activities for her dollhouse.

·        She even often likes playing with the farm animals and the farmhouse.

·        A new idea would be to fill her white tub up with sand and let her play in it with cups and spoons and such.

DAFOs (braces): They give her the usual ankle, arch, and toe support, but they also do not allow her to straighten her knees out all the way because she is at such a risk for hyperextension. We love them. I don’t allow her to do any standing or walking without them on.

Bike and chair: She will sit and stand all on her own. Of course the seat is not too low so she doesn't have far to go to get in an upright position. It's just the right height; she can do it comfortably and build more strength in her legs.

Bike for pedaling: With her sandals strapped to the pedals good, Crystal is now strong enough to pedal the bike on her own. She currently needs to be still taught to continue the pedaling action all the way through to be able to keep the bike going, but this activity is good for the legs, ankles, abdomen, finger grip, and somewhat of the head control.

Bike for scooting: With Crystal’s bottom on the seat, she can reach the floor with both feet and scoot to advance both forward and backwards. She can even turn different directions in the house, basically going anywhere she wants to go. Scooting the bike outside will add an additional challenge to her, but should still be encouraged with a time limit being placed on the activity.

Rifton Gait Trainer: This item is like a “baby walker,” only adapted medically to provide safety for the child who is unable to walk alone. Crystal needs assistance with this item to stop it from rolling out from underneath her and to help her get back up to a standing position when she either chooses to sit or maybe falls.

Toy Rocker: This toy is especially good for abdomen and somewhat of the head control, along with arm strength, and finger gripping. A safety buckle is provided for her protection. She will need help keeping her feet properly placed, but the remainder of the activity she can do on her own.

Walker: This item is used for her own self-security as she advances forward with assisted walking. She can easily push and guide this forward with each step. A sturdy stroller may very well have the same effect.

Stacked Bins: Place 2 sets of white bins, stacked 2 high on each side of Crystal for her to enjoy free play while you are organizing other things for her. This will continue her therapy with abdomen and reaching exercises with an activity she can do without your assistance so you can wrap up other matters that need your attention.

Horse’s Troft (tub): This tub, with her in it, while filling up will provide many areas of therapy to her with excitement and pleasure with little to no fuss. While sitting, it is primarily free play; but as the tub fills and she must be on her knees to stay above water level she will begin meeting several of her needs working areas such as: abdomen, head, finger grip, knee stretching, working back of legs and buttocks, etc. And again as the tub empties, she can go from standing back on to her knees and then there would be no need for her to stay in while sitting.

Bus: This would be one of Crystal’s favorite activities in which we generally have to put a time limit on it because she will choose this activity first and then not want to ever get off. The bus works abdomen, head, lower legs, ankle ROM, but does not include any type of weight bearing.

4-Wheeler: This would be a good alternative to the bus. It provides the same type of workout as the bus but only with it being harder to move it will actually work her more. This activity would also need to be limited because of fatigue and closely supervised for her safety.

Vanity: This is a good table top activity that will cause Crystal to reach a little higher than her usual table with the bins in it to allow imaginative play with table food and/or play jewelry and such. You can use this item while she is standing or sitting at the reddish-orange chair. With the lid closed this item can also be used as a table top for all sorts of table activities like coloring, playing with pegs, playing with babies, etc.

Bicycle with boots: This would be the two-wheeler with the boots screwed to a block of wood, which is then bolted to the pedals. Since this bike’s pedals are straight down from the rider’s hips provided a different type of pedaling work out reaching again different leg muscles. I generally, because of safety issues, put books under the training wheels to elevate the back tire from the ground so she basically rides in place. She has no stability from a fall and must have someone holding her at all times.

Laptop bin: This would be the bin toy that she can have in front of her while she is sitting on the floor. You can put any activity you want in this bin consisting of smaller toys that would provide her free play working her arm and shoulders reaches. She will often push the bin forward using different arm muscles and then she will pull the bin back to her using again a different set of muscles. Sometimes she will even push the bin forward and then scoot up to it.

Laptop activity center: This activity Crystal especially loves for imaginative play using the phone to call friends and family. She adds reaching and mild abdomen and head control. Buttons are good for her finger strengthening.

Go-cart: Dad found the go-cart, Mom supervised the adaptations, and Dad did all the work. She can't pedal to make it move, but we leave her braces on while riding this and the movement of the pedals give her Range of Motion exercises. Also, the movement alone causes her to work her head control and her trunk muscles.

*Special note on all therapy activities: Often Crystal being fatigues and/or tired is a very difficult situation to decide upon. It is not like she says, “I sure am tired.” And she won’t say, “I think I have worked that muscle group long enough.” You may have to watch and get to know her. A big clue would be if she sits not participating in the activity. Whether she is tired, bored, or just not interested in the activity, it would still be best to go ahead and set her up with something else to do. Sometimes she will be cranky or claim she doesn’t want to play with this or that, and that can be both a sign of being tired or not interested. Again, either way, therapy play best benefits her when she is giving full cooperation and participation to the activity at hand and it would be best to remove her from it and try something else. You have lots of choices and lots of different ways to reach all of the different muscle groups, which need to be worked.

VIII. Therapy Toys:
Lite-Brite, Mastermind, and Peg Boards: Are great for pinching. The Lite-Brite will also work her arm and shoulder muscles as she is reaching to place the pegs. This is definitely a table play activity, but can be doubled up with standing, either free standing, in her stander, or even sit/stand in her pink chair.

Wooden Bead Slide, Easels, Connect Four, Magnetic Dolls, and ABC path: These are more examples of reaching activities that generally target the arms and shoulders. They also can be used as table activities while standing or sitting. Often while participating in these types of activities, she will also work the abdomen as well.

Puzzles, Fishing puzzle, Chinese checkers (marble and checkers), wooden blocks, and Shape blocks activities: These activities are examples of what would work on her arms and shoulder muscles in a different way other than reaching. It would be considered endurance in holding the arms up while participating in the activity. A skill she will need to keep strong as possible to maintain best possible ability for her future career and daily functions.

Mr. Mouth game and Frogs: These are two activities that would help with finger stretching and strengthening as she pushes the leverage and then releases. Pushing the leverage down would both stretch and strengthen the fingers. It is best to encourage her alternating with as many of the fingers as possible.

Computer: As Crystal’s disability progresses over the years, she will lose most functions she has today and will probably be limited to working on computers as a career choice. I have not worked with Crystal yet, but I would like to see her be taught and familiarized with the keys, mouse, and functions, practicing using as many fingers as possible on the keyboard and its functions in preparation for her future.

The zoo gazebo: This toy is an excellent tool for working on supination and pronation stretching. If used properly, as she turns the key to unlock the doors, this will utilize her range of motion for her wrist and pivot joint at the ulna and radius.

Clean up: Learning discipline and responsibility is something that we all face while we are growing up and even later as we are adults in our own homes. Crystal is no exception. It is vital that we treat her as normal as possible in every aspect of socially and developmentally growing up. In addition to the need of her learning to clean up her own messes, it is also very good and easy therapy for her. She will have to reach, grab, throw, maybe scoot, and lean somewhat to complete the task of picking up all of the toys. It would be best to make this a routine habit after every activity with little pieces to have her pick up her toys. I believe that for her age, it would be appropriate for the caregiver to assist her until they are all picked up. This includes wiping up spills as well. Having Crystal clean her face and hands is a daily skilled developmental issue that should be practiced anyway along with other skills like getting dressed, brushing her hair, brushing her teeth, etc. She may not be able to do them but she can with your assistance.

IX. Respiratory:
Treatment plan for any coughing-Nebulizer with albuterol, CPT (chest percussion treatments), the in/exsuffalator (cough machine), nasal drops, and syringe the nose free of all reachable secretions (mucous). We apply this treatment plan when she continues to cough for more than a day, even if the cough is a small one.  Even the slightest cough-It does not matter how severe the cough or what the cause of the cough is, the treatment is always going to be the same.  Not only do I want to know if you heard her coughing, I will also want to know: how many times and a brief description of the cough like: how many coughs did that coughing session consist of, was it rattle-like, or short and quick, was it wet or dry.  This is important because it is all of the factors that help determine how soon to start the treatments and how aggressive I need to be with them.

The CPT is applied by first percussing the top front of chest, then top back of chest. Then for drainage purposes we lay her at a 45-degree angle with head down on her side and percuss from lower lung lobes on back to side to front to side to back and repeat for the 2 minutes. Then we flip her and do the other side the same way.

The nasal drops and suctioning of all reachable secretions (mucous) is critical, because she can not expel them on her own, this treatment plan will help with the drainage, but if we don’t pull the drainage out and away from Crystal, it may settle right back into place and our efforts in her respiratory treatments would have been a waste.

Safety-As you pay close attention to any coughing noted it is also important to keep in mind to watch out for any constant, quick, short annoying coughs that she seems to be hung on. Please draw special attention to her: NO MATTER WHAT: if she is sleeping during this time, make sure she is not lying on her back!!! Best plan for this situation would be to lay her upside down (like on her bed), on her side, apply nasal drops, and gently pop her back or front (over the rib cage area including high on the spine just below the neck, listen for nasal congestion, syringe to keep all reachable drainage out and all nasal passageways clear for breathing. Even if she is awake this may help, you may need to do it for a few separate periods of 15 minutes or more each time. Reason: Often, even in the case of a virus or infection, any mucous accumulated or that is trying to drain out will get hung on her vocal cords. The vocal cords are located inside the trachea that leads to the bronchial tubes and on into the lungs. The vocal cords sit about one inch down in the trachea. In order for nay mucous to be coughed up and out of the trachea, the cough must have a good kick behind it. Crystal does not have this power. If the vocal cords get over loaded, they will “tickle” her throat and cause her to cough. If she is lying down on her back the mucous (when the voice box is overloaded) will drain down the trachea into the lungs. WE DO NOT WANT THIS. Any other position and it will not drain this way, which keeps her out of danger but the annoying cough will continue, which is why I suggest the above plan for this problem. Also keep in mind, if she is coughing because of this mucous, it will also interfere and cause complications with her eating, drinking, and swallowing issues. Don’t fret if you don’t see the drainage come out, it can come out the nose, mouth or she can even swallow it. As long as she is not on her back it will not go down the trachea.

Because the body is designed to produce memory cells to help fight recurring viruses and such, we have tried to let Crystal fight any infections using her natural antibodies. Even with the cough and congestion we will let her fight it on her own until fevers reach 100.5 and then at that point we begin her on Augmentin (antibiotic) to help fight the infection in the lungs. It is my belief not to use medicines unless absolutely necessary, however, because she is at such a risk for pneumonia, we watch her very closely and are very aggressive with her care. Before this detailed treatment plan, Crystal had had testimony of developing pneumonia two days after first beginning a very small chuckle type cough and her cough never got so bad that I even recognized it as being any sort of a threat.

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.) 55-80 degrees-May go outside with no limitations 4.) 80- 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.

X. Watch closely for Fatigue:
Consistency is always best with many issues involving these children. As far as things that you would be in control of making sure to avoid fatiguing your child, there are a couple of things that are important:
1.) Make sure that he has a consistent sleeping schedule. For nap and bedtime the same time (or close to it) everyday and make sure you allow him to receive as much sleep as he needs. Avoid situations where you are waking him up. Let him sleep until he is done. If our children do not receive a good sleep schedule then they can stay fatigued for as long as two days after they get back on track with their sleep. I have seen this in Crystal every time I dare to stere away from her schedule she has problem with fatigue and with her it would take her two days to recuperate.
2.) Be sure Crystal avoids heat, in the tub and outside. They both can drain your child of energy and leave him fatigued again for as long as two days. I follow the rules for outside that anything between 55º and 80º Crystal has no limitations on how much time she spends outside. 80º to 90º I only allow her to be out for 15 minutes at a time maybe once an hour maximum. But definitely anything higher than that, then she stays inside. And as far as the bathtub I do not limit her time, I just decrease the heat of the water instead. She has grown accustomed to taking leuk warm baths. I even prepare the water in her horse's troft at a leuk warm temperature. The amount of fatigue acquired from too much therapy has shown in Crystal to be only a few hours though. Often I will put her in her horse's troft a couple of hours before bed and just let her have at it, until she is done. Yes, I see her fatigued when I get her out but I also see in the morning when she wakes up that she is fine, and ready to go for some more. This is also why I rotate her activities throughout the day to different muscle groups, to allow adequate time for her muscles to rest until she works them again. Often while I am assisting her with walking she will ask to sit on my lap and then want to get back up only 2 minutes later.

Why keep her busy?
One important thing that you might want to think about: The only permanent damage in strengthening is the nerve damage. Any nerve cells that may have died while waiting for the diagnosis are dead and there is no way to bring them back. But the muscles are not dead; they are just atrophied. This means no nourishment or no development, which you CAN correct. You just have to nourish them and develop them through therapy and helping your child keep these muscles active. The strength can come back. The control all depends on the remaining nerve cell count. The larger the nerve cell count is the higher range of strength you have to work with. The lower the nerve cell count is the lower range of strength you have to work with. And there is no way to measure this. You just keep working with your child and eventually he will get to where his strength has stopped increasing. That is exactly where the strength gain comes from in these children. They are just strengthening already existing muscles.

XI. Daily Activity Chart:  From top to bottom:

Saturday Activity Chart:  This chart will be the same, with one exception being that from 10 am to 11 am every Saturday, the caregiver will do a thorough range of motion check on all the joints in the body that are listed on the Range of Motion Exercise handout. The purpose of this weekly evaluation is to determine if any additional joints need range of motion therapy to be added to Crystal’s daily plan.

by Brenda Brames

Nadine- Organizing the care, coming up with ideas, constructive time. Almost all my nurses will do my laundry & dishes, but you never know, some of them will not be as apt to do that, especially at first. It's a weird situation for them also. One of the biggest things that I saw that was helpful, one very good nurse we had, was she watched me take care of Jarred & how I did things. You gotta give them a break, they may work with a ton of other families, & they all do different stuff, so it's like learning a new job each new home they get. So I think that is an excellent way to start it off, maybe you could tell them, hey why don't you watch what I do then they can ask ?s etc. instead of starting from scratch. Eventually you learn the strengths & weaknesses of each one & you know who works best with what. I've found my nurses invaluable for PT & also little things like one on one play, that's mostly what it is besides obvious care, it is nice for
our kids that don't have a trach & require too much hands on care, & very different for them if that's what they're used to. It is very tiring at first & you feel like you're invaded in your own home, but it gets easier. I've found talking to them & really getting to know them as a person helps build that trust and also asking about their experience it really helps me feel at ease & helps you to see their heart. It has been a wonderful experience; we get a lot of nursing compared to many of you out there. So I think we're lucky, & we have some of the best nurses:) It is a great experience to say the least and it is great for our kids to have more people to love them. Also, I printed up helpful info like What is SMA etc. from FSMA or something, and stuff like Welcome to Holland & Dr. Bach's protocols etc. for them to read. That is really important to me, to educate them about the protocols and diet & what we do and how vital that is to us.
It will be a relief at least to have extra hands.

Jack Freedman-Sample letter to home health aid:

  Dear Home Care Professional,
Welcome to our home.

Since you are new to the team of professionals who care for our son, I’d like to share some background with you as a way to begin our journey together.

Six years ago when my wife, Anne, and I decided to become parents, we didn’t plan to meet you. Back then, we didn’t know anything about pulse oximeters, wheelchairs, bi-pap (two-way positive airway pressure) machines,
feeding tubes, nebulizers, or standing frames. We didn’t plan for the parade of nurses, social workers, occupational and physical therapists, assistive technology specialists, medical supply personnel, teachers and case managers who come and go. We didn’t anticipate the need for five different doctors to monitor our son’s progress at the local children’s hospital. And we didn’t plan for our child to spend fifty-six nights in the
Intensive Care Unit before he reached his third birthday.

Most of all, when we decided to become parents, we didn’t anticipate that very bad day when a doctor told us our six-month-old baby had a year to live.

So I hope you don’t take it personally when I say I have mixed feelings about your presence in our lives. Frankly, I wish we didn’t need you. I wish our son were going to kindergarten with all the other kids his age, scraping his knees, running around outdoors without his coat on, and
spilling Elmer’s Glue on his lap. I wish I were spending my weekends watching my son play soccer.

But Jack isn’t going to kindergarten, and he isn’t playing soccer. He can’t sit up, walk, or talk very clearly. He can’t move his arms or legs. He can’t dress or feed himself. He still uses diapers. He can’t eat solid foods. He needs constant supervision and attention. He’s medically fragile. His care is physically and emotionally demanding.

I wish we didn’t need you, but we do need you.

We need your positive attitude and your confidence. We need your sensitivity and patience. We need your knowledge, experience, and skills. And we need you to help us have hope, for our son and our family.

When you begin your work with our son, please carry yourself with confidence. You’ve been trained to take care of medically fragile children and we haven’t. Jack’s care requires remembering little things about his needs: what’s safe to feed him, how to lift him into his wheelchair, when
to remove his secretions, how to set up Jack’s arm supports so he can use his computer, which way to turn his head when he lays on his stomach, how often to give him a syringe of formula through his feeding tube.

Although none of this is rocket science, the sheer number of details can feel overwhelming to a newcomer. But if you feel overwhelmed, try not to show it. Act confident. Take initiative. Have a positive attitude. Watch us as we demonstrate how Jack’s equipment works. Ask us questions. Write things down if it helps you. Enjoy yourself when you’re working. But above all, carry yourself with confidence. Jack will feel safe if you feel safe.

When you begin your work with our son, please be patient with us. We may insist about certain aspects of Jack’s care that don’t make perfect sense to you. It may be awhile before we leave you alone with our son even though you’re perfectly capable of taking care of him. You may wonder why we ask you about your own health every time you sneeze or cough in our house. We realize we worry about Jack just about all the time. Please understand that we simply can’t help it. From the moment the doctor sat us down and told us our baby had an incurable, untreatable disease, things were never quite the same again. We really are doing the best we can.

When you begin your work with our son, please be sensitive to our family’s need for privacy one minute and our need for your active involvement the next minute. (As a wise home care administrator I know tells each of her
new clients, “The good news is…you’ll now be getting nursing help in your home. The bad news is….you’ll now be getting nursing help in your home.”). It’s a skill to know when to be involved and when to act invisible. Do your
best to fade into the woodwork when Anne or I need to parent our son. But get right in there with Jack’s physical therapist if she needs your help. Pretend you’re not in the room when I answer the phone and it’s a client emergency. But offer to pick up the phone if it’s ringing and Anne has her hands full with our baby. If you’re not sure which approach to take in a given situation, feel free to ask.

We’ll try to be sensitive to the needs of your family, too. Let us know if you need flexibility with your work schedule because your mother is visiting from out of town or you’d like to attend your daughter’s school play. It feels good to us to help someone else’s family once in a while. We’ll help you out whenever we can.
 
When you begin your work with our son, please take the time to get to know him as a person. Yes, you’re here because of all the things Jack can’t do by himself. But there’s a lot he can do, too. You’ll be surprised the first
time you see Jack drive around independently in his power wheelchair. Try to keep up with Jack as he clicks his way through a tricky challenge on the computer. Feel free to join him in the swimming pool while he works hard in the water with his therapist. Listen to him tell jokes using his
communication device. Enjoy Jack enjoying the company of his baby sister. Soak up his positive attitude, determination, and his smile. We think he’s quite a kid. We hope you will, too. And when you show a genuine interest in Jack, he will fall in love with you very quickly.

When you begin your work with our son, please teach us what you know about caring for him. After Jack’s diagnosis, one of the first things we heard from medical professionals was, “You will soon be the experts on Spinal
Muscular Atrophy (SMA) and caring for Jack.” But I didn’t want to believe our baby had such a terrible disease, much less become an expert on it. And more importantly, if Jack really did have SMA and would die because he
couldn’t breathe, how in the world could we become experts on how to help him? After all, isn’t that what doctors and nurses are for?

Gradually, we did become experts on SMA because Jack is our son and we had to do whatever we could to help him. But for more than five years now, I feel relieved every time we meet a professional who knows more about caring
for a medically-fragile child than we know, or at the very least can teach us something new. As you begin working with our son, tell us what you know, tell us what you’ve learned, tell us what you’ve seen. We take care of only
one medically fragile child.  You’ve taken care of many more than one. You can provide us with some perspective. You can give us a reality check when we need one. You can help us take care of Jack when he’s sick. We need your expertise. That’s why you’re here. Please don’t hold back.

Finally, and perhaps most importantly, when you begin your work with our son, please help us to have hope.

When we first learned Jack was affected by Spinal Muscular Atrophy, hope was a scarce commodity in our home. We didn’t hear much from the doctors about living with Jack from day to day – they didn’t tell us about the good days we would have together as a family. Looking back, I now realize it was Jack’s job to teach us these important lessons. After all, doctors at children’s hospitals don’t spend a lot of time with kids when they are well. Parents do that.

Our son is intelligent and sensitive; he observes all of our words, actions, and signals. Jack needs us to view his disability and his future with a sense of hope, whether life feels manageable at the time or not. Three years ago, when Jack’s bout with RSV led to intubation and a three-
week hospitalization, it was very hard for me to be positive and hopeful. But as I looked at my son lying in the intensive care unit, kept alive by a ventilator, his eyes wide open wondering what was happening to him, I
believed it was important for Jack to see me smiling and to hear me telling stories as usual, giving him the message that I was hopeful and confident of his ability to recover from his illness.

Somehow, Jack did bounce back from that harrowing illness. And ten days later, after he had been successfully extubated and we were preparing to go
home, the head physician in the Intensive Care Unit came by to wish us well. He talked about Jack’s remarkable recovery, and he was obviously pleased to be a part of it. But the doctor’s focus was not on the treatment plan, the efforts of the hospital staff, or his own good work. He
attributed Jack’s recovery to the patient himself.

“Jack’s a fighter,” the doctor told us, quite matter-of-factly.

Indeed, Jack is a fighter. All of the children you care for are fighters. But our children can not fight without hope, and it is up to us – parents and professionals together-- to keep hope alive.

We appreciate everything you do.

Welcome to our home.

Sincerely,
The Freedmans 

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