Expanded Access to OKN-007 for Patients With Diffuse Midline Glioma, H3 K27-altered

Inclusion Criteria:

1. Age from > 12 months to ≤ 18 years of age at the time of enrollment.

2. Diagnosis of diffuse midline glioma, H3 K27-altered according to the WHO 2021Classification of Tumors of the Central Nervous System diagnostic criteria includingdiffuse intrinsic pontine glioma (DIPG). In the absence of a pathologicallyconfirmed diagnosis, a grade IV glioma involving the thalamus, hypothalamus,brainstem, cerebellum, midbrain, or spinal cord, or with a pontine epicenter anddiffuse involvement of the pons.

3. Patient has completed definitive radiation therapy as part of standard-of-caretreatment or was unable to undergo radiation.

4. Karnofsky performance status ≥ 50 for patients ≥ 16 years of age and Lanskyperformance status ≥ 50 for patients < 16 years of age; patients who are unable towalk because of paralysis, but who are upright in a wheelchair, will be consideredambulatory for the purpose of assessing the performance score.

5. Patients must have adequate bone marrow, liver, and renal function:

6. For females of childbearing potential, negative urinary or serum pregnancy test.

7. Patients must be willing to highly effective contraception for both male and femalepatients throughout the treatment and for at least 4 months after last treatmentadministration.

8. Toxicities related to any prior treatments are either stable, stable on supportivetherapy, resolved, or in the opinion of the treating physician, clinicallynon-significant

9. Ability to understand a written informed consent document, and the willingness tosign it. Assent will be obtained when appropriate based on the patient's age.

Exclusion Criteria:

1. Patient is already participating in or qualifies for and is able to enroll in aclinical trial of OKN-007 and other ongoing clinical trials.

2. Patient has received systemic therapy with an investigational agent within 5half-lives or 14 days prior to starting OKN-007 treatment, whichever is shorter.

3. Patients completing radiotherapy treatment less than 14 days prior to planned studytreatment initiation.

4. Current evidence of uncontrolled, significant intercurrent illness that would, inthe treating physician's judgment, contraindicate the patient's treatment withOKN-007 due to safety concerns.

5. Patients who, in the opinion of the treating physician, have not fully recoveredfrom recent major surgery to a sufficient extent to tolerate treatment with OKN-007.

6. Known hypersensitivity to OKN-007 or any component in its formulation.

7. Patients taking prohibited medications as described in the current Investigator'sBrochure.

8. Patient is pregnant or actively breastfeeding.