Inclusion Criteria:

- Genetic Diagnosis: All patients must have confirmatory mutational analysis showing
mutation in the lamin A gene.

- Patients must display clinical signs of progeria as per the clinical trial team.

- Patients must be willing and able to come to Boston for appropriate studies and
examinations at initiation of study and at week 4 of study.

- Patient must have adequate organ and marrow function as defined by study parameters

Exclusion Criteria:

- Other than the drugs used in this protocol, other drugs targeted to treat Progeria
are excluded. Drugs to treat symptoms of Progeria are permitted.

- Patients must not be taking medications that significantly affect the metabolism of
lonafarnib at the time they start lonafarnib.

- Patient must have no uncontrolled infection.

- Subjects who have known or suspected hypersensitivity to any of the excipients
included in the formulation should not be treated.

- Patients must not be pregnancy of breast-feeding. Female patients of childbearing
potential must have negative serum or urine pregnancy test. Male and female patients
of reproductive potential must agree to use a medically accepted form of birth
control while on study and up to 10 weeks after treatment. It is permissible for
female patients to take oral contraceptives or other hormonal methods while receiving
treatment with lonafarnib.