Heads Up On Hypotonia Understanding the Complexities of Hypotonia & Strategies for Treatment By Barbara Hypes P.T. Chapter 5. Strategies List for Treating Hypotonia? 118 • Alignment 119 • Strengthening 129 • Prevent Joint Deformity 139 • Sensory Experiences 142 • Range of Motion 148 • Visual Input 152 • Increase Function and Independence 159 • Personality 161 • Motor Planning 168 • Chapter Five References 175 Chapter 6. Therapy Tools 176 • Toys and Vibrators 176 • Balls 177 • Treadmills/LiteGait 182 • Immobilizers 184 • Electrical Stimulation 185 • Swings, Climbers, and Mobile Surfaces 186 • Wraps 194 • Ambulatory Assists and Devices 195 • Chapter Six References 202 Chapter 7. Common Compensations & Secondary Complications Seen in Hypotonia? 204 • Considering Orthotics 204 • Tip Toe Walking 207 • W-Sitting 211 • Poor Abdominal Alignment 214 • Poor or Absent Development of Head Control 220 • Chapter Seven References 231 vi “Believe nothing, no matter where you read it or who has said it, not even if I have said it, unless it agrees with your own reason and your own common sense.” -buddha Chapter 1: Introduction Frequently Asked Questions • What is Hypotonia? In a 2005 Pediatric Physical Therapy magazine article, Martin et al concluded, “A preliminary characterization of hypotonia indicates that PT’s and OT’s concur that a child with hypotonia displays decreased strength, decreased activity tolerance, delayed motor skill development, rounded shoulder posture with a tendency to lean onto supports, hypermobile joints, increased flexibility, and poor attention and motivation.”1 This conclusion is consistent with what I observe to be true about the way therapists interpret and perceive the delays presented by children who have hypotonia. However, it is not a good description of what I believe are the major challenges of children who present with hypotonia, and it does not begin to explain the complexities of what doctors and therapists call HYPOTONIA. So what is hypotonia? In my opinion, hypotonia is characterized by a depressed ability to organize and use the motor system against the pull of gravity. The person who has hypotonia appears to be collapsed into gravity. This diagnosis typically is determined based on the appearance of the floppy posturing along with weakness, abnormal reflex responses and ligamentous laxity. Many times muscle biopsies, MRI’s, EEG’s and other testing may indicate no recognizable injury to the muscles or brain to explain this apparent collapsed motor control. 5 Experience draws me to speculate that the missing component is a global neurological ability to organize the information from all systems adequately to generate and support typical antigravity motor control. Therefore, therapists often observe that the person who presents with hypotonia has both a primary motor impairment that is recognizable, as well as, or stemming from, a primary neurological organization and integration impairment. If you cannot feel your body or understand how all the parts are connected, you not only find it difficult to move, you may also lack the desire or drive to move. In their 2007 follow- up to the above referrenced article, my interpretation is that Martin et al support this global impairment theory in the introduction stating: “Many disorders, including neurological diseases, endocrine and systemic metabolic diseases, Down syndrome, …Prader-Willi syndrome, cerebral palsy…have hypotonia as a component of the clinical presentation. This partial list of medical diagnoses and disorders that are associated with hypotonia represents a wide variety of etiologies, including central nervous system pathology, peripheral nerve pathology, metabolic disturbances, and hypotonia of unknown etiology.”2 The motor delay of hypotonia may cause a child to feel heavy to carry and actually feel like she/he may slip through your arms. They may have difficulty moving every part of their body or only move with big bursts commonly described as an all-or-none response. The child presents as being very passive, then suddenly appears to have a surge of activation causing them to move an arm or shift or even knock themselves over. When they are positioned and you look at them, they may look poorly aligned as if the bones are not stacked appropriately to look comfortable. The child is heavy to carry because he has not learned how to turn on his muscles to help hold his weight against gravity while he is being carried, thus making him feel lighter. Hypotonic children slip through your hands because their muscles are not active enough to hold the tension as a base for your grasp. They try to move and find it to be difficult so they learn to generate a burst of effort, like we would do when we try to move the refrigerator. The weight of their extremities is as overwhelming to their posture as that refrigerator is to most of us. The burst occurs when they have generated adequate muscle activity to lift or move against gravity. Secondary to their decreased movement experiences, they lack the awareness of how far or fast to lift or move and their lack of consistent experiences further contributes to their inability to grade and control the movement they can initiate. Unlike children with dystonia, when they are assisted to consistently generate muscle activation and holding, people who have hypotonia can learn to hold using graded control and co-activation instead of continuing to rely on their poorly graded bursts to move their extremities or trunk. When they are in supportive positions, the people who have hypotonia may become still and actually sink into the support because they can feel themselves stacked against the surface, and they feel the stablity being provided by the supportive equipment. The processing delay of hypotonia may cause children who have hypotonia to be very irritable and easily upset. Or, they may be “too good.” Babies that are “too good” fail to complain or fuss even to meet basic needs like satisfying hunger. They appear to be content to stay in one spot and look around. They like routines and will actually train 6 their parent to treat them in ways that are routine and predictable so they do not have to experience novelty. They may almost seem to disappear or shut down when things are out of their routine (birthday parties and holidays). Or, they may become overwhelmed and inconsolable so that they can be removed from all the unpredictable newness. The irritability may be a communication that the world is coming at them too fast and out of control for them to be able to decipher any of the details. The “too good” behavior is actually the child slightly shutting down so that she/he can tune out the overwhelming information. But children with hypotonia aren’t necessarily shutting out everything and actually can learn in an apparent withdrawn space, as long as they are not avoiding and turning away the input. Routines are their survival mechanism and they will fuss and complain until caregivers figure out that routine is what makes their children happy and able to function. Although it is great that children can calm down once rigid routines are followed, they begin to be deprived of variety in all kinds of daily tasks because they are demanding rigid predictability. This begins to lead to decreased opportunity for learning at a very young age. When a child can not tolerate being in family gatherings, extended family may resent the child for making it impossible for the parents to attend and they may feel he is spoiled, or a behavior problem even from a very young age. This is true even when the parent tries to explain that the child is obviously overwhelmed by the chaos of family gatherings. One of our most important jobs as infants, besides eating and keeping our airway clear, is modulating state. Infants must learn to move smoothly between being asleep, awake, aware and alert. When they are too unorganized to smoothly make these transitions they may be demonstrating a processing deficit or challenge. When the world is overwhelming in this way, some individuals will withdraw and this posture may look floppy, so these children may initially be identified as having hypotonia. In this example, the motor system is not the primary reason these individuals appear to be collasped in their posture. Instead, their poorly organized or integrated sensory system is unable to make sense of their surroundings causing them to withdraw. These are people who present with hypotonia secondary to sensory system impairments. This book is presented in a effort to define hypotonia as a major sensory and/or motor challenge that can affect development on a continuum, having a mild to profound impact on the individual’s ability to move and interact. It not only affects the motor system but has repercusions on breathing, learning, G-I functioning, visual motor development, independence and accessing the world. It also affects the sensory systems and can affect a persons sense of place and comfort in her/his home, community and in the world. When a person receives no help or is pushed when she should be supported and protected, it can even affect her willingness to stay present and try to overcome the complexities of interpreting the information surrounding her. 7 Fig. 17 Fig. 18 Fig. 19 In Figure 17, the child is able to lift her arms and head using good alignment in the neck and shoulders because the girdle of proximal control is adequate to allow this lift when the resistance of gravity is minimal. In Figure 18, the head is lifted laterally and the child is using co-activation in her neck and left shoulder to maintain this aligned lift now that the resistance of gravity is stronger. In Figure 19, the child is horizontal to the resistance of gravity and her proximal stability is overwhelmed by the weight of her head staying lifted against the pull of gravity.