The word arthrogryposis comes from the Greek derived "arthro-", joint + "gryposis ", crooking. The word “multiplex” is a Latin derived word meaning “many” or “multiple” and “congenita” is a Latin derived word meaning “existing at or dating from birth”. Therefore, the term Arthrogryposis Multiplex Congenita can be loosely translated as "lots of crooked joints at birth." These joint contractures develop before birth (prenatally) and are evident at birth (congenitally). A newborn with arthrogryposis lacks the normal range of motion in one or more joints. It is really more of a description than a diagnosis, as most people have AMC as a result of another condition.

Joints affected by AMC may include the jaw, spine, shoulders, elbows, wrists, fingers, hips, knees, ankles, feet and toes -- virtually any and all joints.

The range of motion in the joints of the arms and legs is usually limited or fixed. The impairment of joint mobility in AMC is often accompanied by overgrowth (proliferation) of fibrous tissue in the joints (fibrous ankylosis).

In normal embryonic development, the joints begin to develop by about 5 to 6 weeks of gestation. There are joint spaces by 7 weeks, and the limbs can be seen moving by 8 weeks. This motion of joints is clearly essential to the proper development of the joints and structures around the joints. Limitation of fetal motion/joint motion before birth leads to joint contractures.

Prenatal limitation of joint mobility can result from:

ALL ABOUT UPPER EXTREMITIES  

Common Upper Extremity Deformities
Internal Rotation of the Shoulder
Extension of Flexion Contractures of the Elbow
Flexion Contractures of the Wrists
Thumb in Palm
Extension or Flexion Contractures of Individual Fingers

Courses of Treatment

Therapy

O/T should begin as soon as possible to increase passive range of motion. This can be done through a clinic or at home with your local Early Intervention. Either way you should be taught stretches to do at home with your child. Here are some common stretches to try. Always speak to your O/T or Child’s Doctor before starting any kind of new routine.

Stretches

Thumb- This is hard when your baby is very tiny. Put your thumb in the palm of your child’s hand and push outward on the thumb at the base of the hand. Be very careful not to just push on the thumb itself because you can dislocate the thumb at the joint that connects it to the hand.
Fingers- If in flexion straighten out each finger and hold. If in extension gently curl finger. Be very careful and do not go beyond your child’s limits. Do not force anything or you can break their little bones.
Wrists- This is also a very hard stretch on very tiny babies that are very stiff. Place your thumb under the center of you child’s wrist and push upward while bracing the top of their wrist with your middle finger straight across.
Elbows- Be sure you find the elbow joint and are bending in the correct direction. This may sometimes require rotating the arm externally a little bit. Bend the elbow very slowly and gently. As with any other stretch be very aware of how your child is feeling. You know the difference between the annoyed whimper and the cringe of pain. Don’t go any further than your child will tolerate.
Shoulders- This can vary a lot depending on the actual quality and condition of your child’s shoulder joint. I would consult an O/T before doing any kind of shoulder stretches.

Stretches no matter what kind are very important and should be done at least 6 to 8 times a day. A lot of parents do stretches at every diaper change. It makes 1 less thing to remember!

Splinting

Splinting is used less often then therapy. A lot of doctor’s believe they can restrict movement and sometimes become more cumbersome than helpful. Though not always effective, splints provide a sustained stretch. They are most often prescribed for over-night wear. Most often they are wrist splints used to slowly stretch the wrists out of flexion. There are many types:

Benik- Soft neoprene splints with a metal bar that can be manipulated to give different amounts of stretch
Custom Made Splints- These are fabricated by an O/T or Orthotist. They are usually made with the same material as AFO’s and can be custom made to your child’s hands. They are secured most often with velcro straps.

Elbow splints are prescribed less often. I have not ran across any commercially manufactured wrist splints that are small enough for children. Often an O/T or Orthotist can fabricate these using the same splinting material used in AFO’s or wrist splints. They then attach velcro and a rubber band that connects to a wrist splint and they provide a sustained stretch while child is relaxed. When the child wants to they can extend their arms it just takes work for them to do so. The other down side to this is that it actually strengthens the muscle that extends the arms, by giving the child the option to extend their arms if they push out. Though this does not usually outweigh the benefits.

Surgery
Often with children who have little passive elbow flexion, surgery is recommended. Doctor’s often recommend an elbow release surgery between 8 months and a year. This is a surgery done by releasing the tight fibrous structures in the elbow that constrict movement. After that if is child still does not develop any active flexion or enough functional passive flexion doctor’s may recommend a muscle transfer. This depends on the quality of the donor muscles as well. Common muscles used are the pecs and a muscle from the back, Latimus Dorsi. Most doctor’s will not perform this surgery on children under 4 or 5, but every doctor is different in how aggressively they treat upper extremity problems. Some doctor’s do not believe this surgery is effective at all and do not even offer this as an option to their patients. There are also several hand surgeries that can be performed. For children with a serious flexion contracture in their wrists a small triangular wedge of bone can be removed to bring the wrist to a more neutral resting position. There are also surgeries for thumb in palm, if extremely severe, and various finger surgeries.

This info sheet is not complete - feel free to add to it as you see fit

Info compiled by Wendy

ALL ABOUT LOWER EXTREMITIES (coming soon)

EARLY INTERVENTION

Early intervention services help young children with disabilities achieve their goals in cognitive, social/emotional, communicative, adaptive, and physical development. Services may include occupational therapy (OT) to work on upper extremities/fine motor, physical therapy (PT) to work on Lower Extremities/gross motor skills, or speech therapy (ST)  to help a child learn to eat, speak, and improve oral motor skills.  Service Coordination through a local agency (ex: Department of Mental Health, MRDD, and Department of Education) is provided as well. Some states also offer developmental therapy (DT) provided by an Education Specialist or Developmental Therapists who track general developmental progress and work on pre-academic skills.  Most early intervention services take place in the child’s Natural Environment; this could be the child’s home, daycare facility, or babysitter.  Parks and other community facilities are also considered a part of the child’s natural environment. 

Early Intervention services are crucial to the healthy development of children with disabilities.  Through the years, early intervention services have proven to be vital to the healthy development of infants and toddlers with disabilities, minimizing their potential for developmental delay. With early intervention, the child will decrease the range of delay when compared to their peers.  Early intervention helps to reduce the need for special education and related services once the children reach school age.  This lowers the educational cost to local schools.

If a child is determined through a Multi Factored Evaluation* (MFE) to have a developmental delay, the EI program works with the family to develop an Individualized Family Services Plan (IFSP).   *(A MFE assesses a child’s development in all or some of the following areas: fine and gross motor, speech, cognitive abilities, adaptive skills, and social skills).

The IFSP is a written document developed by a team of individuals including the child's parents and representatives from the state's early intervention program (OT, PT, ST, DT, service coordinators etc.). The IFSP includes statements about:

• The infant/toddler's presenting levels of performance based on objective criteria;
• The family's resources, priorities and concerns regarding the development of the child;
• Measurable goals the child is expected to achieve as a result of receiving services; how these goals will be measured, timelines for achievement and methods to modify goals as needed;
• The specific services needed to meet the individual child and family’s needs clearly stating: frequency, minutes per week/month and delivery method;   
• The natural environment where services will be provided, or the reasons why services will not be provided in a natural environment;
• The dates for start of services and the length of services;
• The service coordinator that will be responsible for implementation of the IFSP and coordination with other agencies and persons; and
• Steps necessary to support the child’s transition to preschool or other appropriate services when appropriate

Congress created the Early Intervention Program for Infants and Toddlers with Disabilities to help families whose children have special needs.  This program is under the umbrella of IDEA and is Titled:  Part C. If you feel that your child has a developmental delay, ask your pediatrician about your state’s EI services. Some pediatricians want to wait until children are six months old before referral, if this occurs, you may need to initiate the process yourself.   Each state runs their program a little differently, so calling the state coordinator’s office would be a good place to start.    The National Early Childhood Technical Assistance Center offers links to each state’s Part C Coordinators.  Each state’s office should be able to direct you to your local coordinator.  http://www.nectac.org/contact/ptccoord.asp  click on PartC and then select your state.  

Info compiled by Michele

TYPES OF THERAPY 

Aqua Therapy

Why AQUA-THERAPY?
Aquatic Rehabilitation is fast becoming a leading therapeutic exercise alternative. Water is an ideal treatment medium for patients with painful joints and weak muscles, allowing for earlier intervention. The buoyancy of water dramatically decreases stress on weight bearing joints, bones and muscles. In addition, water promotes general muscular relaxation and provides consistent resistance throughout a range of motion while also offering support for injured or otherwise weak areas, greater tolerance of activity, and easier gains in range of motion with less pain.

Patient populations benefiting from this form of therapy include those with:
conditions including: multiple sclerosis & arthritis
Joint Disorders/Replacements
Orthopedic Injuries including
Shoulder
Hip
Knee
Ankle
Back
Chronic Pain
Post Surgical Debilitation
Loss of Motion
Muscle Weakness
Fibromyalgia

The benefits of AQUA-THERAPY are:
Decreased pain
Increased mobility
Increased strength
Improved coordination
Increased muscular endurance
Increased relaxation
Increased flexibility
Improved posture
Improved cardiovascular status

The physical properties of water and their effect on the human body help to explain the benefits of aquatic therapy (hydrotherapy.) Water's buoyancy virtually eliminates the effects of gravity - supporting 90 percent of the body's weight for reduced impact and greater flexibility. For example, a 140-pound woman weighs only 14 pounds in water. Water acts as a cushion for the body's weight-bearing joints, reducing stress on muscles, tendons and ligaments. As a result, aquatic workouts are low impact and can greatly reduce the injury and strain common to most land based exercises.

Due to viscosity, drag forces and frontal resistance, water provides a resistance which is proportional to the effort exerted against it. Resistance in water ranges between 4 and 42 times greater than in air, depending on the speed of movement. This makes water a natural and instantly adjustable weight training machine. Unlike most land based exercise, water provides resistance to the movement in all directions which allows all of these directions be used in the strengthening process. Water's resistance can be increased with speed and/or surface area and the resistance is proportional to the effort required to move against it.

The unique properties of water enable your heart to work more efficiently. The hydrostatic pressure of water pushes equally on all body surfaces and helps the heart circulate blood by aiding venous return - blood flow back to the heart. This assistance to the heart accounts for lower blood pressure and heart rates during deep water exercise versus similar exertions on land.

Movement and resistance properties allow patients a great deal of control...the patient is in charge! The greater the speed of movement, the greater the resistance and vice-versa.

http://www.stpta.com/aqua_therapy.htm
http://www.indialife.com/Health/aqua_therapy.htm
http://www.rehaboutlet.com/1004_1.htm

Info compiled by Jenilee

HippoTherapy 

• Abnormal tone
• Impaired balance responses
• Impaired coordination
• Impaired communication
• Impaired sensorimotor function
• Postural asymmetry
• Poor postural control
• Decreased mobility
• Limbic system function related to arousal, motivation, and attention

• Gross motor skills such as sitting, standing, walking
• Speech and language abilities
• Behavioral and cognitive abilities

•  Cerebral Palsy
• Cerebral Vascular Accident (stroke) 
• Developmental Delay
• Down Syndrome
• Functional Spinal Curvature
• Learning or Language Disabilities
• Multiple Sclerosis
• Sensory Integrative Dysfunction
• Traumatic Brain Injury
• Autism 

Hippotherapy or Therapeutic Riding: What is the difference and how do I know which one is the most appropriate for my child?

     1). Consider your child's specific needs:

• Does your child require constant positioning to maintain sitting balance?
• Does your child need frequent assistance to maintain attention or alertness levels?
• Is your child under the age of 5?
• Does your child have special medical needs that may require the additional knowledge and training from a licensed professional therapist?
• Does your child have sensory integration dysfunction or frequent behavioral outburst to sensory stimulus?
• Does your child have specific neuro-motor goals to work on?
• Would your child benefit most from the horse's movements in private 1:1 sessions?

If you answered "yes" to any of the questions above, then hippotherapy may be the avenue most appropriate for your child at this time. 

     2). Next, look at the availability of programs within an hour drive of your area.  Often there are many riding programs available but few therapists offering hippotherapy.  In addition, therapeutic riding lessons may be offered seasonally and/or programs may have waiting lists.  These are important factors in your final decision.

• To find a center near you, see the directory at NARHA, the North American Riding for the Handicapped Association

   3).  And finally, consider the costs involved.  Riding lessons typically cost less than professional treatment, however, therapy fees may be covered under your health insurance policy.  You will need to consult the programs and professional in your area for more information related to your particular situation. 

More about the American Hippotherapy Association

Formed in 1993, the American Hippotherapy Association's mission is to promote research, education, and communication among physical and occupational therapists and others using the horse in a treatment approach based on principles of classic hippotherapy.  Registered therapists in hippotherapy are located throughout the United States and Canada.

Sites used to provide this information:

http://americanequestrian.com/hippotherapy.htm

http://americanhippotherapyassociation.org/aha_hpot.htm

http://rightsteptherapy.com/hippo.htm

http://narha.org

Info compiled by Jen (Isabel's Mommy)

Physical Therapy 

Physical therapy also begins very early. A lot of doctors prefer aggressive physical therapy prior to any splinting or surgery. They want to see how much motion can actually be gained without the big ordeal of surgery. Most parents are taught physical therapy to begin immediately with their child. These stretches are very important and are done many times a day in the first few months. Many children are also referred to a physical therapist through EI or their hospital. Physical therapists will also start working on stretches with your child as well as other activities to help them stay on track developmentally. They will try to help you figure out ways that your child can do things, how ever they need to be modified. They will focus on gross motor skills first such as rolling, sitting, getting to a sitting position, crawling, standing, walking, and walking up and down stairs. They will also help your child get use to using any assistive devices your child needs such as crutches, a gait trainer, or walker. Later on in your child’s life they can also assist with the transition to school by helping your child practice skills they will use in school- sitting in a desk, walking down the hallways, playing outside. This is just a very general outline of physical therapy. Each therapist will tailor your child’s course of treatment based on your child’s abilities and what they need to work on as well.

Occupational Therapy  

Occupation Therapy? Sounds pretty funny for a child, huh? Your child’s occupation is performing activities of daily living at age appropriate levels. These are the things he or she does to “occupy” his or her time. A lot of time upper extremities take the focus in occupational therapy as much time can be focused on self feeding (when age appropriate), dressing skills (also age appropriate), and fine motor skills. Occupational therapists are also trained to work on “stretching” or increasing range of motion (active or passive) in your child’s joints. They may also be the one who will assist you will arm & upper extremity splints. Later in your child’s life you occupational therapist may also help your child with writing and other fine motor skills needed for school and other activities of daily living. Occupational therapist may also assist in ordering adaptive devices for all of these activities such as special feeders or writing equipment. Finally, occupational therapy done early on is definitely believed to help children gain the greatest amount of function possible to perform activities of daily living.

Speech Therapy 

Not all children with Arthrogryposis require speech therapy, though it often begins a very young age in these children. Some children with Arthrogryposis have physical reasons such as a small palate, small esophagus, and generally weak muscles including those used for chewing. Other children with require speech therapy because of oral sensitivity issues such as textures. Yet, others will require speech therapy due to a delay in actual speech. At a young age they can begin to work with children on allowing different textures such as rubber teethers and small vibrations in and around their mouths. At older ages they can work with children and textures of food. They also can work with children on many aspects of speech such as understanding (receptive speech) and vocalizing (expressive speech). They can also help with breath control and some other weakness related issues that affect speech. Speech therapists vary the techniques they use very much and sometimes even the most normal activity can constitute a therapy session. Often times speech therapists will read to a child or sing to a child and try to get them to sing along as well.

Info for PT, OT, & ST compiled by Wendy (PJ's Mommy)

Cranialsacral Therapy Coming Soon!

TYPES OF AMC (coming soon!)

AMYOPLASIA
DISTAL
NEUROGENIC
TrismusPsuedocamptodactyly Syndrome

GENETICS (coming soon!)