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Hip Instability In Patients with Down Syndrome

In the fall of 1998, our daughter was diagnosed with hip dyplasia and subluxation, ultimately requiring surgery (Ganz periacetabular osteotomy) with hip spica casting in November of 1999. Although we were fortunate enough to take Ashley to a medical facility considered to be one of the finest in the world, it was disappointing to us how little information appeared to be available to parents about hip dysplasia, hip dysplasia in patients with Down syndrome, and issues related to the surgical procedure and postoperative care.

As a service to others, we will be including a number of things which we hope will be helpful to you or someone you know who may be faced with this diagnosis and/or the surgical repair for hip dysplasia. The areas covered include the following:

Abstracts
Web Sites
Books
Key Words and Definitions
Prior to Surgery
During Hospitalization
At Discharge
Transportation To and From Major Medical Centers
Caring for a Patient in a Hip Spica Cast
Cast Removal
Physical Therapy

Kimberly S. Voss
Harold M. Voss, Jr., M.D.

Note: We have run across a list of surgeons who perform periacetabular osteotomies. We are not endorsing the list or any particular surgeon but are providing it for informational purposes only. 8/24/08


Abstracts

In the Health Care Guidelines for Individuals with Down Syndrome: 1999 Revision (Reprinted from Down Syndrome Quarterly, Volume 4, Number 3, September, 1999), it states that "Interestingly, congenital hip dislocation is not commonly encountered. Hip dislocation is more often seen in the older child and the adolescent."

Studies we reviewed showed that from 7.9% of patients with Down syndrome to 28% of adults with Down syndrome had some hip abnormality, while hip subluxation occurred in 4% to 5% of patients with Down syndrome. But, interestingly, it was surprising how little information was retrieved when a literature search was executed. For this search, the words "hip instability and Down syndrome" were used. To view these abstracts, click here.

For additional free searches of MedLine using the online service PubMed, click here.


Websites

Dislocation of the Hip in Children with Down Syndrome
Pediatric Development and Behavior: Congenital Hip Dysplasia
Wheeless' Textbook of Orthopaedics: Down Syndrome: Orthopaedic Considerations
Wheeless' Textbook of Orthopaedics: A New Periacetabular Osteotomy for the Treatment of Hip Dysplasias

For a National Association for Down Syndrome (NADS) discussion board regarding hip problems and Down syndrome, click here and click here.


Books

Medical & Surgical Care for Children with Down Syndrome: A Guide for Parents published by Woodbine House addresses the "Orthopedic Issues Affecting Children with Down Syndrome." There is a small section which deals with the "Hip Conditions" of individuals with Down syndrome which includes congenital hip dislocation, acetabular dysplasia, and "otherwise normal hips that tend to dislocate."



Key Words and Definitions

To assist in understanding the often foreign sounding language used by doctors and other healthcare professionals, we have included terminology which you may run across when experiencing medical care or surgery regarding hip dysplasia and/or hip subluxation.

acetabulum - The cup-shaped socket of the pelvis into which the head of the thigh bone (femur) fits.
ambulate - To walk.
analgesia - A state of insensitivity to pain though usually fully conscious.
anesthesia - Loss of normal sensation or feeling, and usually consciousness, without the loss of vital functions. This state is produced artificially by a drug or gaseous agent.
atelectasis - The collapse of all or part of the lung. Atelectasis is commonly seen immediately in the postoperative period in patients who have undergone general anesthesia. Symptoms of atelectasis include low-grade fever (<101 degrees), dry cough, chest pains, and mild shortness of breath. Additionally, atelectasis may be manifested by a lower than expected oxygen saturation level on pulse oximetry. Mild postoperative atelectasis is treated with deep breathing exercises, upright positioning, ambulation, and respiratory therapy.
atrophy - A wasting away or decrease in size of a cell, tissue, organ or part, usually brought on by disuse.
capsular plication - To decrease the size of the joint space membrane (capsule) by removing the excess tissue.
CPT - Chest physiotherapy, a form of respiratory therapy.
decubitus ulcer - A break in the skin that appears in pressure areas in debilitated patients confined to bed or immobilized, due to a circulatory defect from the enhanced tissue pressure in high-contact areas, often occurring over a bony prominence.
dislocation - The displacement of any part from its normal position or location, typically referring to bones.
diuresis - An increased excretion of urine.
dysplasia - Abnormal growth, development or formation.
edema (edematous) - An abnormal accumulation of fluid causing puffiness and swelling. This is commonly seen postoperatively.
epidural analgesia - The use of narcotic and/or local anesthetic injected into the area immediately outside the spinal cord membrane (dura mater) to achieve good pain relief with minimal sedation. This process involves the placement of an epidural catheter in the lower or mid-back.
febrile - Related to or characterized by fever.
femur - The thigh bone.
osteotomy - The surgical cutting of a bone.
PCA - Patient Controlled Analgesia. A pump device that delivers pain medication intravenously. This device has the ability to deliver a "basal" rate, i.e., a rate continuously administered without any action by the patient. The device also has the ability to deliver a "trigger" dose, i.e., an additional amount of pain medication when the patient presses a button. These modalities are prescribed by your physician, and the basal and trigger dose may be adjusted as necessary.
PCEA - Patient Controlled Epidural Analgesia. A pump device that delivers pain medication and/or local anesthetic through an epidural catheter. This device has the ability to deliver a "basal" rate, i.e., a rate continuously administered without any action by the patient. The device also has the ability to deliver a "trigger" dose, i.e., an additional amount of pain medication when the patient presses a button. These modalities are prescribed by your physician, and the basal and trigger dose may be adjusted as necessary.
periacetabulum - Around or near the hip socket.
pulmonary toilet - Maneuvers to assist the lung in eliminating secretions and mucous to lessen the risk of a lung infection or pneumonia, such as CPT, breathing treatments, and positioning.
pulse oximeter - A monitoring device used routinely during and immediately after surgery which uses an infrared light to detect the % of maximum possible oxygen carried in the blood.
PRN - As needed.
PICU - Pediatric Intensive Care Unit.
PT- Physical therapy; or, an individual who is trained to use exercise and physical activities to condition muscles and improve level of activity.
respiratory therapy - Exercises and treatments that help patients recover lung function, such as after surgery.
spica (hip spica cast) - Casting which is opened around the buttocks and perineal regions for elimination and hygiene, used for treatment of congenital hip dislocation and for immobilization after reconstructive surgeries. A "full spica" cast involves both legs. A "half spica" cast involves one leg.
spinal analgesia - Also known as intrathecal analgesia. The use of narcotic and/or local anesthetic injected into the spinal fluid around the spinal cord and inside the spinal cord membrane (dura mater) to achieve good pain relief with minimal sedation. This process usually involves a single injection of medication without a continuous catheter as in the epidural route defined above.
subluxation - The abnormal movement of one of the bones that comprise a joint; not a true dislocation but a partial dislocation.
urinary catheter - Often referred to as a "Foley" catheter after the original brand. This is a hollow tube passed into the bladder which allows the urine to drain from the bladder freely into a bag.

For additional definitions to medical terminology, check the On-Line Medical Dictionary, a searchable online dictionary of medical terms.


Prior to Surgery

By attempting to anticipate needs prior to surgery, the surgery and hospitalization can proceed more smoothly, providing a better transition to healthcare and recuperation at home. Being prepared and exhibiting a willingness to participate and ask questions of individuals involved in your child's care are also characteristics of being a good advocate.

Educational Services: Make arrangements for educational services that are anticipated to be necessary following surgery. By doing so prior to surgery, your time and attention can be focused on the medical care and recuperation of your child rather than battling with the school regarding the amount and/or type of service delivery. By waiting until after surgery to address these issues, you may run the risk of the services no longer being necessary by the time an agreement can been reached.

Discuss with your child's surgeon the anticipated length of time your child will require adjustments in her school day and provide all necessary documentation to satisfy your child's school district. If homebound services are required and your child is on an IEP, the IEP must reflect this "change in service delivery" or "change in placement." By anticipating the maximum amount of time services may be needed and reflecting this in the IEP, you may avoid the necessity of an additional IEP meeting during your child's recuperation to extend the time required for services, such as homebound services. And remember: there is no "standard procedure" within a school district for such services; frequency and duration of services are determined on an individual basis.

Home Health Care: Discuss with your surgeon the anticipated home health care needs of your child. Contact your health insurance provider to determine what may or may not be covered, including durable medical equipment (such as wheelchair, walker, hospital bed, bedside toilet, etc.) and nondurable medical equipment (such as diapers, "blue pads", etc.). Also, inquire about possible home health care assistance. Ask your health insurance provider what documentation is necessary to acquire these items or services so that this may be taken care of in a timely manner.

Purchases: Consider purchasing all anticipated nondurable medical equipment prior to surgery (shampoo tray, diapers, "blue pads," etc.). This can all be waiting at home following surgery. Additional bed pillows and blankets are also excellent for propping and positioning. Extra bed sheets also come in handy as "draw sheets" for positioning and for anticipated changes from soilage.

Transportation: Begin to explore the various options for transportation to and from the medical facility. These decisions will be closely dictated by whether or not casting is necessary, as well as the distance traveled, and the age, development, and size of your child. Ask your surgeon whether or not casting will be required, and discuss whether or not various options for transportation warrant consideration.

Housing: If the surgery occurs outside your hometown and housing is necessary during the hospitalization, consider making reservations in a wheelchair accessible hotel room. It is possible that you will be discharged from the hospital to this room before you are ready to head home. You will need the extra space for the wheelchair, transfers, etc.

Also consider checking on the possible availability of a Ronald McDonald House in the city in which the surgery will occur.

Support: Speak with other families who have been through the same or similar surgical procedure. They will not only provide invaluable expertise but also the emotional support to help you get through this very challenging period.


During Hospitalization

Consider the following:

• Have a family member or friend present in the room with your child at all times.
• Ask what is being administered each time medical personnel are hanging IV fluids, or giving medications. IV bags can be checked to make sure the proper patient's name appears on the bag.
• If casted, check for areas of discomfort or redness which may be caused by the cast and/or long periods of immobility. Adjustments may be made by the surgeon to the cast by trimming the problem area(s) or adding additional padding. This is important to avoid the development of pressure sores.
• If cast petalling is to be done (placing overlapping waterproof tape over the cast edges to help keep the cast clean and dry, and to avoid rough edges), be sure that this is taken care of prior to the removal of the urinary catheter.
• Although hospital personnel will be frequently checking your child's various vital signs, you may also check your child's toes for color, movement, sensation, and warmth. Toes should remain warm and pink with good blood return.
• Do not be alarmed by swelling/edema in the genital area. This is not unexpected due to the proximity of the surgical incision to the genital area. Ice and elevation may reduce the swelling and discomfort associated with this.
• Discuss the plan for pain management for your child during their hospital stay, that is, whether it is on a schedule or on an "as needed" (PRN) basis. This plan should be individualized depending upon such things as your child's age, disability, cognitive skills, type of surgery, and expected level of pain.
• Do not hesitate to ask medical personnel for instructions in making proper adjustments to the hospital bed to assist in your child's comfort.
• Do not hesitate to remind medical personnel about other medical issues your child may be dealing with. While hospital personnel may be focused on healing one specific area of the body, they may inadvertently neglect taking into consideration other preexisting conditions.
• Because bedside toilets will probably have to be brought from the hospital's "central supply," anticipate its need by making sure that it is in the hospital room and immediately available to your child.
• It should be anticipated that physical therapy will be involved in your child's care during their hospital stay. Be sure that you feel properly instructed on and comfortable with positioning and transfers, a necessary skill during your child's recovery at home.
• Ask your physician the expected or approximate time of hospital rounds for the following day so that you may be present to discuss your child's status and care.
• If appropriate, ask that hospital social services be contacted on your behalf. They are there to provide assistance with the hospitalization and discharge planning.

But most importantly, realize that you know your child better than anyone. Note things that are of concern to you, or changes you have observed; write them down, and feel empowered to discuss them with your child's nurse and/or physician.


At Discharge

Leave the hospital with the following in hand:

• prescriptions for all necessary medications (possibly including but not limited to pain medication and stool softeners)
• prescriptions for necessary durable and nondurable medical equipment, possibly including but not limited to a hospital bed (with or without a trapeze), bed side toilet, wheelchair (possibly reclining), and walker
• prescription for any necessary home health care
• a list of physical restrictions
• a list of symptoms which should precipitate a phone call to the physician (such as, swelling, high fever, constipation, etc.)
• a schedule for follow-up doctor's appointment(s); this may include the appointment for the cast removal at which time an additional prescription for physical therapy may be given
• a letter giving permission for travel by commercial airline, air ambulance, etc., if applicable
• a letter for school to provide homebound services and some indication of duration of need for services if this has not already been taken care of

Discuss with your physician the benefit of receiving physical therapy during the early recuperation (nonweightbearing period) at home. A physical therapist can assist in instructing direct care providers with transfers and positioning in the home, as well as provide direct therapy to your child to avoid atrophy of the unoperated leg and upper body. Since a walker may be the next mode of ambulation following a period of nonweightbearing, maintaining the use and strength of the arms will be important.

Consider securing items which you may need in a hotel room prior to departure for home, or items that may be needed in transit. These might include the following:

• roll of waterproof tape for petalling the cast
• diapers and pads
• "blue pads"
• bed pan


Transportation To and From Major Medical Centers

Because the surgery to correct hip dysplasia might have to occur at a major medical center rather than a community hospital, travel arrangements may become an issue. Transportation options can be directly affected by the necessity and extent of casting. If a hip spica cast is necessary, the joints of the affected side(s) are so limited by the casting that the patient will be unable to walk. Additionally, the angle at which the hip and knees are casted will also affect how erect the patient can become while sitting and the extent to which the casted leg(s) extends from the body.

Initially, the casting for our daughter was such that she could not sit at more than a 45 degree angle, precluding most travel options and leaving us with an air ambulance as our only apparent alternative. But once a "window" was cut away in the chest area of her cast before being discharged from the hospital, she was able to sit in a more erect position, allowing us to return home on a commercial airline in first class bulkhead.

Again, these decisions will be closely dictated by whether or not casting is necessary, as well as the distance traveled, and the age, development, and size of your child.

Air Ambulance

Although an expensive option generally not covered by medical insurance, an air ambulance can place patients on a plane using a stretcher. But whether necessary or not, some form of medical personnel will fly along as well, at least partially accounting for the high cost.

The cost of an air ambulance is determined by a number of factors including distance traveled, number of necessary medical personnel, and number of passengers (patient and family members). Some air ambulance costs are all inclusive, providing all transportation from "door to door," possibly including an ambulance or stretcher van which may be unnecessary. Be sure to compare bids carefully.

Charter Aircraft

Charter aircraft may not be an option if the patient is nonambulatory (unable to walk). Charter services that we contacted indicated that passengers had to be able to board the plane with "minimal assistance," excluding it as an option for someone in a hip spica.

If a charter service is found which will take a patient with a hip spica, be sure to inquire about the type of equipment to be used for reasons of accessibility.

Angel Flight

Angel Flight is a non profit charitable organization providing air medical transportation for patients and their families. Be sure to check on the type of equipment to be used for reasons of accessibility.

Angel Flight (corporate headquarters)
Angel Flight, Inc. (Oklahoma)
Angel Flight Central
Angel Flight East
Angel Flight South Central

For additional charitable transportation resources, check the following:

Mercy Medical Airlift
National Patient Travel Center

Also visit the Family Village for more resources.

Commercial Airline

While traveling by commercial airline may be an option, careful consideration must be taken for the length of travel, layovers, changing of planes, and toileting issues during the period of travel.

It is best to try to avoid as many transfers as possible. We were able to use our personal wheelchair to our seat in first class bulkhead on the commercial airplane, avoiding the transfer to the small "dolly" used by the airline to take nonambulatory passengers to their seats.

Be sure to have a letter from your doctor giving permission to travel by commercial airline. This may be requested from you at the time of check-in.

When arranging seat assignments, bulkhead is preferable, and first class may be necessary. An aisle seat will, in all likelihood, be necessary. It is preferable to seat the patient on the side of the plane where the operated side of the body is toward the window, especially if the leg has been casted. Otherwise, the casted leg may be sticking out into the middle of the aisle.

While the airline will tell you there is room on board for personal wheelchairs, we have not found this to be the case. Although individuals traveling in wheelchairs typically board first and storage areas should not yet be taken by other passengers' belongings, we have found that airline personnel (pilots, flight attendants, etc.) store their bags in the area where wheelchairs could be placed. Instead, wheelchairs are more often "broken down" and stored in the belly of the plane with other luggage where they are "last on" and "first off." You should receive a claim tag for the wheelchair. Wheelchair components may be placed in the overhead compartments of the plane.

Stretcher Van

Stretcher vans can be used to travel longer distances than an ambulance, yet do not provide the level of care or cost of an ambulance. But there is typically a limitation to how far they can travel in a given day so this should be explored if it is a great distance to travel home.

Personal Vehicle

Depending upon the distance to travel home, it may be possible to use a personal vehicle, such as a van or mini van, to transport your child to and from surgery. Seats could be removed and a mattress placed in the back of the van or mini van. Be sure to take toileting issues into account when making your plans. 


Caring for A Patient in a Hip Spica Cast

Personal Hygiene, Bathing, Hair Washing

Sammons Preston for "Special Purpose Products for Enhanced Living" has a limited web site. But their catalog contains a number of shampoo trays and other products for daily living which may be helpful. Visit their web site to receive a catalog.

Functional Solutions for "Products that make performing everyday activities a little easier." Their catalog carries shampoo trays and other products for daily living.

Toileting

For additional information on caring for a patient with a hip spica cast, check out the following web sites:
Hip Spica Cast: A Guide for Patients from the University of Iowa's Virtual Children's Hospital
Hip Spica Cast Care from the Cincinnati Children's Hospital Medical Center
Care for a Child with a Hip Spica Cast from the University of Chicago Children's Hospital Pediatrics
The Do's and Don'ts of Cast Care from the University of Chicago Children's Hospital Pediatrics


Cast Removal

A special "saw" is used to remove the cast. Consisting of a vibrating disk, it cuts through the casting materials before stopping at the padding. The sound of the saw itself is quite loud and very intimidating.

Consider doing the following:

• Show your child how the saw works prior to removing the cast.
• Bring along a "walkman" type of CD player with headphones which could be used to distract your child and muffle the sound of the saw.
• Take along extra sets of hands or ask for additional assistance in the doctor's office.

The removal of the cast proved to be one of the more unpleasant aspects of this entire experience for Ashley. Ashley was initially very lethargic once her cast was removed which was cause for some concern. It was anticipated that she would experience muscle spasms and was given pain medication to alleviate her discomfort.


Physical Therapy

Physical therapy will, in all likelihood, be an essential component of recovery from orthopedic surgery of this nature. Before surgery, discuss with your surgeon the role the physical therapist will play, including whether the physical therapist will be able to initially provide therapy in the home setting rather than in a clinic setting, and whether physical therapy will be utilized prior to the removal of the cast or splint while the patient is nonambulatory.

A physical therapist can be a tremendous help with the recovery from hip surgery, including:

• teaching techniques for making transfers
• providing guidance regarding hip precautions
• choosing and properly fitting the patient with a walker and/or wheelchair
• assessing barriers in the home for a walker and/or wheelchair, and
• providing transition planning and instruction to the school physical therapist.

When Ashley's cast was removed and she was allowed to begin some weightbearing, she was initially rather put off by using a walker. But once her physical therapist added a basket to her walker and introduced a few games she could play with things carried in her basket, she was more willing to learn to use it. She became quite proficient with its use and went nowhere without it for many months.

While the length of recovery is different for all patients, Ashley's recovery was an intensive 6-7 month period following her surgery. Physical therapy moved from twice a week to once a week, to once every other week, to no therapy until school services picked up a small amount of direct service. The goal from her surgeon was that she continue physical therapy until she returned to at or near her level of functioning prior to her hip surgery. But Ashley probably did not progress like a typical patient without disabilities recovering from a similar surgery since she was not only compromised by her cardiac status but was also compromised by the low muscle tone associated with Down syndrome, the challenges to her gait before and after surgery, the atrophy from the casting, as well as the nature of the recovery from an orthopedic procedure of this complexity.

Ashley did not return to school full time for the duration of the school year but continued to receive home instruction, as well as attending school a couple of hours a day 2 days a week with Mom present.

She ultimately required her right shoe being built up by an orthotist to compensate for a leg length discrepancy as a result of the surgery. This appears to have been caused by a change in the height of the hip socket rather than a bone length discrepancy.


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