Who Is the Oldest Progeria Survivor?

1. Chad Wedekind (1973 to 2012)
2. Margaret Casey (1956 to May 26, 1985)
3. Leon Botha (June 4, 1985 to June 5, 2011)
4. Sammy Basso (December 1, 1995 to present)
5. 5. Michiel Vandeweert (June 13, 1998 to present)
6. Claudia Amaral (1999 to present)
7. Mickey Hays (June 30, 1972 to June 30, 1992)
8. Devin Scullion (August 1996 to January 23, 2017)
9. Megan Nighbor (June 2000 to present)

Progeria, also referred to as Hutchinson-Gilford syndrome, is a rare, progressive genetic condition that causes children to age rapidly during their first two years of life. The appearance of progeria in children at birth is generally normal. Signs and symptoms, such as slow growth and hair loss, begin to appear during the first year.

Strokes or heart problems are the most common causes of death in children with progeria. Approximately 13 years is the average lifespan of a child with progeria. The disease can cause death earlier or later in life for some individuals, whereas others may live longer, up to 20 years in some cases.

Although progeria cannot be cured, research suggests there is some promise for treating the disease.

Hutchinson-Gilford progeria syndrome is a genetic disorder characterized by rapid and dramatic aging at a young age. The affected child looks normal at birth and early infancy but grows more slowly than other children and does not gain weight at the expected rate (failure to thrive). Their facial appearance includes prominent eyes, a beaked nose, thin lips, a small chin and protruding ears. It also causes hair loss (alopecia), skin with aged appearance, joint abnormalities and a loss of fat under the skin (subcutaneous fat loss). In this condition, there is no effect on intellectual development or motor skills such as walking, standing and sitting.

As early as childhood, people with Hutchinson-Gilford progeria syndrome develop severe hardening of the arteries (arteriosclerosis). Heart attacks and strokes are much more likely to occur at a young age due to this condition. Affected individuals may experience serious complications that can worsen and become life-threatening.

The growth of a child with progeria slows significantly within the first year of life, but motor development and intelligence remain normal.

Symptoms of this progressive disorder include

• Slowed growth, with below-average height and weight
• Narrowed face, small lower jaw, thin lips and beaked nose
• Head disproportionately large for the face
• Prominent eyes and incomplete closure of the eyelids
• Hair loss, including eyelashes and eyebrows
• Thinning, spotty, wrinkled skin
• Visible veins
• High-pitched voice

These signs and symptoms also include progressive health issues, such as

• Severe progressive cardiovascular (heart and blood vessel) disease
• Hardening and tightening of the skin on the trunk and extremities (similar to scleroderma)
• Delayed and abnormal tooth formation
• Partial deafness (dysacusis, hypoacusis)
• Loss of fat under the skin and loss of muscle mass
• Skeletal abnormalities and fragile bones
• Stiff joints
• Hip dislocation
• Insulin resistance

Typically, progeria is recognized at an early age, usually during regular checkups, when the symptoms of premature aging begin to appear in infants.

If you have any worries about your child's growth or development, or if you notice any progeria-related symptoms in your child, please consult your child’s doctor.

Progeria is a rare, progressive disease caused by a single genetic mutation. This condition involves the gene LMNA (lamin A), which produces a protein known as progerin, which is responsible for maintaining the structural integrity of the cell's nucleus. When a mutation occurs, the protein becomes defective, causing the nucleus to become unstable. Due to the instability of the cell structure, progeria is characterized by early and rapid aging.

There are several possible complications, including

• Atherosclerosis: Hardening of the arteries that restricts blood flow
• Heart problems: Congestive heart failure and heart attack
• Cerebrovascular problems: Lack of blood supply to the brain causes strokes
• Arthritis: An inflammatory condition affecting one or more joints and causing pain or stiffness
• Cataract: Clouding of the clear eye lens

The condition is diagnosed based on signs and symptoms and a physical examination. Physical examinations include

• Taking the child's height and weight
• Checking eyesight and hearing
• Blood pressure measurement

Genetic testing can confirm LMNA gene mutations. Because progeria is a very rare medical condition, it is likely that the doctor may have no experience treating or diagnosing it. As a result, if progeria is detected in a child, it is imperative to speak with the doctor about a treatment plan.

Currently, there is no cure for progeria. To detect cardiovascular disease at an early stage, it is necessary to monitor the heart regularly. Additionally, parents need to take their children for regular checkups, where the doctor plots a growth chart based on the child’s height and weight. In addition to checking the child's eyesight, hearing, and dental health, the doctors will examine the child's overall health.

There are several treatment options available to relieve some signs and symptoms of progeria, including

• Aspirin: May be prescribed to prevent heart attacks and strokes.
• Statins: Used for lowering cholesterol levels.
• Antihypertensives: Reduce blood pressure.
• Heparin or warfarin (anticoagulants): Prevent blood clots in the body.
• Antiepileptic medications: These medicines prevent seizures.
• Physical and occupational therapy: Physical and occupational therapy helps the child function normally and relieves joint stiffness.
• Nutritious diet: The ideal diet for maintaining adequate nutrition is high-calorie meals with supplements.
• Dental examination: Because this condition results in delayed and abnormal tooth development, regular dental examinations are necessary.

To treat progeria, more investigation or research is needed

• On gene mutations to uncover the cause of this disease and develop new treatments.
• To develop new methods of preventing atherosclerosis.
• To further study the effects of drugs belonging to the group of farnesyltransferase inhibitors, such as lonafarnib, which can help gain weight and make blood vessels more flexible.
  • Zokinvy (lonafarnib) capsules have been approved by the FDA to decrease the risk of death from Hutchinson-Gilford progeria syndrome.

The following considerations should be kept in mind while helping a child with progeria:

• Keep the child hydrated as dehydration can lead to fatal complications. Make sure the child drinks enough water during the summer and when sick.
• Provide the child with high-calorie foods and supplements.
• Consuming small, frequent meals will increase the child’s calorie intake.
• Make sure that the child maintains an active lifestyle following a doctor's consultation.
• Have your child wear cushioned shoes or shoe inserts.
• Make your child wear sunscreen with at least 15 SPF.
• Make sure your child gets all vaccinations on time.