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Popliteal Pterygium Syndrome
What is popliteal pterygium syndrome?
Popliteal pterygium syndrome is a condition that affects the development of the face, skin, and genitals. Most people with this disorder are born with a cleft lip, a cleft palate (an opening in the roof of the mouth), or both. Affected individuals may have depressions (pits) near the center of the lower lip, which may appear moist due to the presence of salivary and mucous glands in the pits. Small mounds of tissue on the lower lip may also occur. In some cases, people with popliteal pterygium syndrome have missing teeth.
Individuals with popliteal pterygium syndrome may be born with webs of skin on the backs of the legs across the knee joint, which may impair mobility unless surgically removed. Affected individuals may also have webbing or fusion of the fingers or toes (syndactyly), characteristic triangular folds of skin over the nails of the large toes, or tissue connecting the upper and lower eyelids or the upper and lower jaws. They may have abnormal genitals, including unusually small external genital folds (hypoplasia of the labia majora) in females. Affected males may have undescended testes (cryptorchidism) or a scrotum divided into two lobes (bifid scrotum).
People with popliteal pterygium syndrome who have cleft lip and/or palate, like other individuals with these facial conditions, may have an increased risk of delayed language development, learning disabilities, or other mild cognitive problems. The average IQ of individuals with popliteal pterygium syndrome is not significantly different from that of the general population.
How common is popliteal pterygium syndrome?
Popliteal pterygium syndrome is a rare condition, occurring in approximately 1 in 300,000 individuals.
What genes are related to popliteal pterygium syndrome?
Mutations in the IRF6 gene cause popliteal pterygium syndrome. The IRF6 gene provides instructions for making a protein that plays an important role in early development. This protein is a transcription factor, which means that it attaches (binds) to specific regions of DNA and helps control the activity of particular genes.
The IRF6 protein is active in cells that give rise to tissues in the head and face. It is also involved in the development of other parts of the body, including the skin and genitals.
Mutations in the IRF6 gene that cause popliteal pterygium syndrome may change the transcription factor’s effect on the activity of certain genes. This affects the development and maturation of tissues in the face, skin, and genitals, resulting in the signs and symptoms of popliteal pterygium syndrome.
Wheelchair Accessibility
Wheelchair accessibility helps people who can no longer get around without support. You can optimize your home and your daily life to make things easier for your loved one if they are not as mobile as they use to be.
Wheelchair Ramps
Ramps make it much easier for people in wheelchairs to exit and enter their homes. The material for the ramps should be standard wood, but you can use protective coatings on the ramp to make sure that the weather doesn’t weaken it. You must check to see if the person using the wheelchair can easily push themselves up the ramp and down the ramp without the wheels getting snagged on anything.
Don’t space the wooden planks too far apart. The gaps in the wood can cause a bumpy ride at the least and a health hazard if you’re not careful. You can even buy portable ramps that you can take with you on road trips. The person in the wheelchair may also need help getting into vehicles and other establishments. Portable ramps can certainly come in handy at the most inconvenient times.
Wider Doors Inside
You must ensure that doors have easy paths of travel. Don’t place boxes or other items close to doors when a person using a wheelchair will have to navigate through the opening. The doors in the home may need to be wider if they are less than 32 inches wide. The wheelchair user must have plenty of space to get through.
Wider doors can also make a people feel more comfortable inside the home. The bare minimum amount of space can make them feel cramped and closed in. They should at least be given some freedom of movement even though they are using a wheelchair.
Wider Hallways
Wider hallways are also essential to the comfort and well-being of someone who gets around in a wheelchair. The wheelchair must be able to move freely through the halls with plenty of space to spare. Make sure there is at least 36 inches of space between the walls in the hallways.
You might also need wider hallways if the wheelchair will need to turn corners to get to different rooms. It can be difficult for a wheelchair to navigate through tight corridors without rounded corners. Leaving plenty of space will ensure a happier and healthier experience.
Bathroom Changes
It’s time to get rid of that bathtub in your bathroom. Showers are much more accessible for wheelchairs than bathtubs. You can also install hand-held shower heads and seats so that people using wheelchairs can have a better chance at bathing themselves. The person will be much more comfortable in the shower since they can just open the door and wheel their way in.
Buckle Up!
No matter how the wheelchair is secured to the vehicle, a properly used and positioned crashworthy seatbelt, consisting of pelvic and upper-torso belts, is absolutely essential. Seatbelts are by far the most effective occupant restraint system for protecting occupants in crashes and reduce the risk of total injuries by more that 50%. They prevent occupants’ ejection from and minimize injurious contact within the vehicle.
To be most effective, the lap belt must be placed low on the pelvis near the top of the thighs, and the shoulder belt should cross the middle of one shoulder and the breastbone and connect to the lap belt near the occupant’s hip.
While wheelchair securement and occupant restraints are important, a growing body of evidence suggests a large proportion of serious injuries to wheelchair-seated travelers is due to a lack of proper seat belt use and/or improper positioning of the seatbelt. In many cases, wheelchair features such as armrests and wheels can interfere with proper seatbelt routing and placement, and care must be taken to ensure that seatbelts are properly used and positioned. This may require placing the lap belt between the back of the armrest and the seatback post, or threading the lap belt’s end through openings below the armrest before attaching the belt to the vehicle’s anchor points. It is also important to place the seatbelt buckle in direct contact with the occupant and not where it may contact rigid wheelchair components during a crash. Never route seat belts outside the large wheels or over armrests.
Even if you are only driving a short distance under ideal conditions, fasten your seat belt.
You should never drive until everyone has a seat belt properly fastened.
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