Our general attitude became – if it is NON-INVASIVE and can’t hurt, then LET’S TRY IT! It took us awhile to arrive at this position with Dylan’s care, but it would have been nice to realize it and be able to articulate it from the beginning.
A note of encouragement regarding all therapies: None of the therapies are “rocket science” and anything beneficial needs to be incorporated into your child’s daily routine. You are now your child’s therapist. If a therapy is helpful to your child, then chances are, once per week treatment is not enough (and that is often what insurance will pay for). Make sure you ask your doctor and/or therapist first about the frequency. Then watch your therapist carefully, ask lots of questions, and take notes. Learn from the therapist how to care for your child, practice WITH the therapist, and then do the activities recommended by your doctor and therapist yourself on non-treatment days. Your child will benefit. Also, you will feel good about the fact that you can help your child. Again, therapies give you an opportunity to spend more time ‘loving on your child’, will make your child feel better, and will do the same for you BECAUSE you are helping your child. Touch is an essential human experience and another valuable way to show love for your child.
Your child’s therapists can become almost a part of your family. We found that we loved anyone who loved our Dylan. The therapists typically have a huge place in their hearts for special needs children. We formed close bonds with these people who were with us day in and day out, and many of those bonds continue to this day. In fact, there are three professionals who are contributing to this website who are like family to us. They have contributed their time and talents to this project to honor Dylan, whom they still love.
This is a difficult thing to say. It is important to realize that care and therapy for a terminal child are different than care for a child with a 20-80 year life-expectancy. There are things that must be done to preserve muscles and organs for 50 years that are not necessary for a 5 year life expectancy. Quality of life must always be considered. There are some therapies that are just not worth the pain and struggle for the child to go through. However, there are things that should be done to preserve a child’s functions should he live, as Dylan did, far beyond the projected life expectancy. Most physical therapists are not accustomed to working with children who are terminal. You might have to “rein in” your therapist if they get “greedy” with their expectations. This is a conversation that we have had multiple times with different medical professionals. The mindset needs to be balanced – “we need to best preserve the function of our child’s mind and body, while allowing him to enjoy the time he has on earth with our family”. There is not a black and white answer here, but you may need to articulate to your therapists that there are things you do not want to do because they are too invasive or painful for the potential benefit your child will receive. The role of your therapist is to provide information and expertise, but you are the ultimate decision-maker about your child’s care.
(Per consultations with Sandra Zaccari, Physical Therapist, and Mindee Jones, Occupational Therapist). Therapy is an important part of your child’s overall medical plan. There is also a large overlap between physical therapy and occupational therapy. Many issues are addressed here which can fit in either discipline, and your therapists can hopefully work together for the best fit for your child, based on their individual expertise. Outlined below are some of the areas that should be addressed in your child’s care. Your therapist(s) will work with you to develop an individualized therapy plan to meet your child’s specific needs, which will change over time.
It is important to consider whether or not your therapists are specifically trained in pediatrics. There are numerous differences between therapy for children and adults and a pediatric therapist has expertise that is important in working with children who have developmental delays. In addition, they are more familiar with the necessary equipment for children with special positioning needs. Don’t hesitate to request a pediatric therapist, even if your agency has to outsource for services. (Our hospice organization hired a couple of our therapists as contract labor, at our request).
See below for further information on OT.
Maintaining the flexibility of the joints and muscles (complete range of motion) throughout the body is important for several reasons. First, it prevents contractures of the joints and tightness of the muscles that can lead to problems with mobility or positioning. Attempts to move joints that are tight can be painful as well. Second, movement provides your child with an awareness of his body in space and can increase levels of alertness. Third and most importantly, touch is one of the most essential human experiences and is another valuable way for your child to know how much they are loved.
Therefore, it is important to have the child participate in range of motion exercises daily. This can be done through play (as he is able), or by being passively stretched by another person. Make sure to range every joint, taking care to have one hand support the joint you are working on. For example, to range the shoulder, place one hand on the shoulder while the other hand is helping to raise the arm in the air. Please consult your child’s PT or OT for more information on the proper technique.
Weight-bearing is important because it builds stronger, denser bones.
Arms – to encourage weight-bearing through the arms, the child can lie on his tummy on the floor while looking at a book or playing with a toy. Another way is to lie over an exercise ball and push up through the arms while watching TV, or anything that engages his attention.
This became one of the positions Dylan hated. Screaming while lying on his tummy during a photography session was the very first inkling we had that something might be wrong. We thought it was just because he hadn’t gotten enough tummy time on the floor (since we had two older, rough boys and a dog). Dylan didn’t tolerate therapy for weight-bearing (and by not tolerating, we mean crying and letting us know he hated every second of it) until I tried holding him on my stomach. He would then push up on his arms to make eye contact with me while I did goofy things.
Ensuring that your child has proper positioning is essential for several reasons. It helps maintain their postural alignment, assists with use of the arms, as well as helping in proper breathing and digestion. The rule of thumb to aim for is sitting in a 90-90-90 position, which means the hips, knees and ankles are all bent at 90 degree angles. As your child becomes less active, it will be critical for you to frequently change their position in order to prevent pressure areas and joint/muscle tightness. Make sure to change positions at least every 2 hours to avoid bed sores (common pressure areas include buttocks, heals and elbows).
Depending on your child’s current level of ability, positioning devices can be as simple as pillows, or more adaptive such as foam wedges.
It is important to keep the shoulders supported when in a stationary position for any length of time. If not, the arm can pull out of the socket (subluxation). Regardless of the position (laying down, sitting up, standing), it is important to make sure the arms are supported on a surface (tray, lap pillow, rolled towels).
It is also important to find a balance between proper positioning and comfort. The best position is often not the most comfortable. However, you don’t want to sacrifice longer-term function of your child’s body for shorter-term comfort. As an example, we used a hammock for short periods of time and Dylan seemed very comfortable. The hammock does not provide great support, so we used this for short periods. See Photo #9 below.
If your therapist is not familiar with the equipment options, you can contact the medical equipment vendors directly. The vendors can often do in-home assessments and measuring/fitting for adaptive devices.
a Recliner – this was a favorite place for Dylan. Be sure to use pillows and rolls to provide good positioning and alignment. Sitting in a recliner for too long, however, can contribute to rounding of the spine, which was a problem for Dylan. Try to get his back as straight as possible, while supporting the arms under the elbows, to prevent subluxation. See Photo #5 below with Dylan in his favorite recliner.
b Bean Socks – these are tube socks filled with dried beans. We used them for many purposes in positioning, because they have more support than pillows. (See Sleeping, below).
c Neck pillows with beads – we used these for Dylan while he was in his stroller and recliner to get his head upright. See photo #6 below, showing Dylan in one of his strollers, with a neck pillow.
d Boppy Pillow – These can be a great support tool for positioning because they are firmer than pillows. They can be lap pillows in the front while sitting. You can also use one in the front and one in the back to support sitting.
e Weighted blankets - These are useful because the deep pressure can be calming. It can also reduce the infant startle reflex. The rule of thumb is that the blanket should be 10% of the child’s body weight. If you are using a smaller pad, (such as a lap pad), the typical rule of thumb is 5% of body weight (because it is not covering the entire body). These can be purchased online or you can make them yourself from online directions. They can be filled with a variety of objects, such as beans or popcorn, which make them heavy.
f Sleeping – Dylan slept on his side with his head on a pillow. We put bean socks behind his back so he wouldn’t roll backward. We propped his arm onto a pillow to keep his shoulder comfortable.
g Versaform Chair - this is a bead filled square bag that has air in it. When the air is sucked out with a pump, the chair becomes firm (hard as a rock) and conforms completely to your child’s body shape. It provides ideal comfort and positioning; however, it takes at least 2 people to get it set up. The therapist (either PT or OT) and I would use our bodies to hold Dylan in the position we wanted him in, then one would work the pump to suck the air out of the bag. We would then prop the chair against a piece of furniture at a comfortable angle. See the Equipment section of this site to find Versaform pillows/chairs. See Photo #7 below – an example of Dylan’s Versaform chair, covered in a blanket.
h Tumbleform- This is a solid piece of vinyl-covered foam with a separate base. The chair can be positioned on the base in various reclining positions depending on your child’s head and trunk control. This is ideal for use as a feeding seat also. It comes in 3 different sizes. See Photo #8 below – showing Dylan’s Tumbleform used in a swing.
i Traditional Strollers: These can be used if your child still has fairly good head and trunk control. A five-point harness is helpful for positioning.
j Adapted Therapeutic Strollers: There are many types and styles available and your therapist should help determine the best fit for your child’s needs.
k TLSO: TLSO is an acronym for Thoracic Lumbo/Sacral Orthosis. As a child loses trunk control, his spine may become rounded, with a risk of possible scoliosis. If left unattended for too long, a permanent curvature of the spine could occur that could compromise breathing and digestion. If a child is positioned well, this can usually be avoided. However, if necessary, the PT and orthopedic doctor can determine if this type of brace would be helpful in maintaining proper spinal alignment. It can be worn for a few hours at a time, or longer depending on the deformity. It can also be used as support for sitting activities if the child still demonstrates head control.
l Benik Vest: The Benik Vest is a soft neoprene vest that provides compression as a form of sensory input, but no real support of the spine. See the Equipment section of this site for access to Benik vests.
m AFO’s : This acronym stands for Ankle/Foot Orthoses and refers to braces that are worn to provide support at the lower leg for standing activities, or to prevent contractures of the ankle/foot. In a relaxed position, our foot tends to point downward. Without opportunities to bear weight, the calf muscles can become tight and contracted. This can impact sitting positions in a stroller with a footplate or any weight-bearing in standing. It also impacts comfort.
n Knee Immobilizers : As mentioned previously, knee immobilizers are soft knee braces with embedded metal stays and Velcro closures that can be used to provide support of the legs for assisted standing, or to help stretch tight hamstring muscles.
See the "Equipment" section of this site for further information.
(Per consultations with Mindee Jones, Occupational Therapist)
1) Feeding – factors such as decreased oral motor control, decreased strength and endurance, as well as aspiration can affect the quality and safety of eating. Here are some tips to make feeding a safer process. Some of these tips might be valuable even in children with feeding tubes, as it can help them handle their secretions better.
a Oral Motor Stimulation: apply pressure around and in the mouth before eating to “awaken” the muscles needed for chewing. Some tools that OT’s use for this are: nuk brush, jiggler, z-vibe, P and Q rings, electric toothbrush (if tolerated) or regular toothbrush. Also, to clean the mouth after eating, apply pressure around the mouth with the cloth rather than swiping lightly across the face. This will increase “oral motor input” and may help the child tolerate this often non-preferred task as well.
For Dylan, we tried the infant finger toothbrushes for oral stimulation. We found that he would bite down very hard and it really hurt! We tried a feeding nipple from a Farmer’s Co-op (not for calves as they are too big – it may have been for a lamb?) and it worked better because the rubber was much thicker. Obviously, we sterilized it first.
b Positioning for eating: If the child has decreased endurance and strength to maintain an upright position, it is important to make sure he is seated with good support before eating. It can also help prevent aspiration by having his head, neck, esophagus, etc. in the proper alignment. Tucking the chin when swallowing can also help. Adaptive equipment such as a Tumbleform chair (see Equipment) or seats with a five point harness are helpful in maintaining good alignment for feeding.
c Aspiration: If your child is coughing a lot during feeding, he may be aspirating. Aspiration occurs when the food “bolus” or liquid is going into the lungs instead of the esophagus. This can lead to pneumonia (which is the most frequent cause of death in Krabbe children). Consult a doctor, speech therapist and/or occupational therapist who is skilled in feeding issues for further guidance. Your child might benefit from having a Barium swallow test to determine what consistency of food or drink is needed to ensure success. Often thickening the liquids and pureeing the solids will help.
2) Bathing is an obvious necessity, but not necessarily an easy task. This is an “activity of daily living” (ADL) that your Occupational Therapist can help you with. Mindee was present many times during Dylan’s bath to help us “work out the kinks”. There are many bathing chairs available in therapy catalogs and on the internet. See the Equipment section of this site for further information on bath chairs.
Dylan hated the cold temperature of the air during his bath. We found it helpful to place a space heater in a small bathroom to warm up the whole room before, during and after his bath. We also kept a hand towel draped over whatever part of his body was not submerged. We frequently poured warm water over him. Dressing also provides a challenge, because when a child is upset or cold, he tends to draw his limbs in. We laid a fluffy towel on the floor, then covered him with a second towel. While dressing him, we kept a towel covering whatever part of his body didn’t have clothes on yet.
3) Dressing also provides a challenge, because when a child is upset or cold, he tends to tighten his limbs, which may already be tight anyway. Dressing your child where it is warm (especially after the bath) can be helpful to prevent the cold, startle reflex that can cause him to go into extension, making it harder to bend his arms and legs to get into the clothes.
4) Sibling Interaction: Involving the siblings in therapy sessions (as tolerated by all parties!) is a great idea for many reasons. The siblings can feel useful and important to the fragile child AND their parents. It can increase bonding between the siblings. It can provide crossover – the siblings can do things with the fragile child throughout the week that is just “extra” therapy. The fragile child just wants to be a regular kid, included and part of the family. Any kid-to-kid interaction is a beautiful thing for fragile children. It can also stir an element of compassion in the siblings that is impossible to teach without “living” it.
5) Sensory Integration has many benefits. Sensory Integration refers to the ability to take in sensory input, interpret and react to it. Some people crave more input, some want less and prefer to avoid it. Understanding your child’s sensory needs can provide comfort by decreasing sensitivity to noxious (or OBNOXIOUS) stimuli. Providing sensory input that he tolerates and enjoys that addresses each of the senses can be therapeutic and helpful in improving his quality of life.
The following are areas that affected Dylan:
a Vision---he was sensitive to bright lights and benefitted from wearing sunglasses or a hat to shade his eyes; we provided him with visual tracking exercises and used light box therapy to provide visual stimulation he could tolerate. Also consider keeping lights low in your home or use lower wattage light bulbs.
b Auditory---unexpected, loud sounds were startling and would cause him to become tense and upset; music often relaxed him.
c Deep pressure---Dylan was comforted by deep pressure. Massage, weighted blankets, and Bean Socks are some things that can provide this input. The touch should always be firm, not light or tickly.
d Vestibular---At different stages, Dylan tolerated gentle, linear movement applied by either rocking gently on an exercise ball or from swings. This was relaxing for him and also provided good stimulation. We hung a doorway bar swing with a large hammock net swing (placing a bean bag or large pillow or blanket for him to lie on helped aid in his comfort); We also had an adapted swing that he could use when outside.
e Olfactory---certain smells can be obnoxious, however several can provide comfort (such as lavender) or are tolerable stimulating smells (such as cinnamon). You can find further information on aromatherapy online or ask your OT.
6) Play Therapy/Music Therapy: To maintain quality of life, it can be important to continue to offer the children opportunities to engage in play. It also can help motivate them to participate in activities that help range their muscles, aiding in maintaining good joint mobility, endurance and strength. The type of play depends on each child’s age and abilities. Cause and effect toys that are easily activated are often effective. In Tennessee, the Technology Access Center can adapt toys/electronics for free for families. For example, children can activate a bubble machine, turn on a fan or radio, or watch Elmo do the Chicken Dance simply by touching a large button.
C) Chiropractic Therapy – Dylan saw a Chiropractor at least on a weekly basis for his entire illness. He had a significant curvature to his spine that we felt must be uncomfortable. The chiropractor used a Percussor to stimulate muscles and for relaxation. For a long time, the chiropractor came twice/week to use the Percussor on Dylan. We were able to justify to our insurance company that it would be cheaper for them to purchase the Percussor for us so we could use it on him daily, and cut back the Chiropractor to once each week. The Occupational Therapist and Physical Therapist were then also able to use the Percussor on Dylan as part of their treatment. See Equipment Page for Percussor Information.
D) Traditional Music Therapy – We tried some music/play therapy that was incorporated with Occupational Therapy. We also tried a professional Music Therapist, which was more play therapy that involved music. Also, there was a hospice volunteer who provided music therapy for Dylan. He sang and played guitar to whatever kind of music that Dylan enjoyed (mostly relaxing songs). It was very sweet. Dylan often fell asleep during the music, which made us believe he enjoyed it (or at least found it relaxing).
E) Whirlpool / Water Therapy – It seems that many Krabbe children find a whirlpool bath relaxing. It is definitely good for muscle relaxation. For Dylan, we found that stripping him down, then the shock of the water, and the struggle of getting dressed again undid most of the benefit he had received. However, we did find that Dylan enjoyed the pool (IF the water was quite warm), as this was more of an extended period of time. We built a Pool Chair which used a modified Bath Chair for use in the pool. See the Equipment Section regarding Bath Chairs and the Pool Chair.
F) Massage Therapy – we tried but were unable to get massage therapy approved by our insurance or Hospice. We felt it was beneficial and important, so we tried to compensate by using the Percussor (discussed at Chiropractic Therapy above) and in other therapies. Dylan did not like any kind of “tickle” touch, but only firm, warm touch. When massaging, lotion is helpful, but it needs to be warmed in the hand first. Many muscles will get tight (especially the hamstrings and inner thighs), so it is important to keep them loose in whatever way works for your child. See also Botox injections in the Medical Section, for tight muscles.
G) Chest Percussion Therapy (CPT) (Respiratory) : This sounds very official and complicated. In fact, it is quite simple. Your physical therapist and/or nurse can show you how to do it. In hospitals, it is done by a Respiratory Therapist. There is a little thumper (rubber cushiony device) that you gently thump on your child’s chest and back in a pattern. This serves to loosen potential congestion in the lungs. In our case, we burped Dylan frequently (during and after all meals and bottles). We used a cupped hand that simulated the thumper and got double-duty out of the burping. Since we did this so often (at least 4 times/day), it could have been beneficial in fighting off pneumonia during the years. Strangely, Dylan seemed to enjoy this and was relaxed by the process.
H) Cranial Sacral Massage Therapy – This is a light pressure to the skull performed by a licensed therapist. It is very relaxing, non-invasive, can’t hurt and was part of our Hospice Service (which we requested). Here is a more official description: “The practitioner gently works with the spine and the skull and its cranial sutures, diaphragms, and fascia. In this way, the restrictions of nerve passages are said to be eased, the movement of cerebrospinal fluid through the spinal cord is said to be optimized, and misaligned bones are said to be restored to their proper position.”
It is helpful to involve everyone on your child’s “team” in a periodic meeting. We wished we had thought of this earlier in Dylan’s life, but we felt the whole team benefited from the several times we met together. Perhaps not every person can make it, but you can consider asking the following: Doctor, Nurse, OT, PT, Chiropractor, Special Needs Educator, Social Worker, Grief Counselor, etc. We asked each team member to think about an item they would like to see “crossed over” to other disciplines (such as Percussor treatment by the Chiropractor used by the Occupational and Physical Therapists). Then an information / cross-over training can be conducted right there in the group. Goals for your child can be set and everyone can get on the same page to ensure their discipline contributes to the goals. For example, if dressing is becoming difficult due to tight arms, the OT, PT and Chiropractor might all decide on some stretches they can each do at their sessions. Also, professionals in a room may brain storm ideas that would not come up in an individual setting.