Fast Facts updated 03/04/04
Benjamin Charles Stokman - Birth on 10/23/01Diagnosis
Symptoms
- Ben went to the hospital because his eyelid swelled shut.
- We learned he had a serious problem through a MR scan of the brain, the start of seizures and loss of vision and the growth of scalp lesions.
- We diagnosed the disease through skin and brain biopsies.
- Ben was Diagnosed with JXG (Juvenile Xanthogranuloma), a rare disease.
- We have been doing everything in our power to learn about this disease and its treatment.
- The disease is similar to another 'histiocytosis' called LCH (Langerhans Cell Histiocytosis).
- We do not know what causes histiocytosis such as JXG.
Treatment
- Ben has brain, lung, kidney, scalp and skull bone (orbit) involvement.
- In hindsight, Ben's eyelid swelled shut because of his lesion in his orbit bone, above and outside his left eye.
- He has lesions or tumors in several locations, the ones in his brain are of the greatest and most immediate concern.
- Ben started with 4 lesions in his brain, he now has 8. The largest is over an inch long.
- For a time the brain lesions were growing at a high rate (10% in 10 days), in June '02 they seemed to slow down or even stop, in July '02 they have grown about 10%. Over the many months since he started 2-CdA they tumors appear to have stopped growing.
- Ben's scalp lesions worsened slowly while on his first chemo (Methotrexate, Vinblastine & Prednisone), but on the 2-CdA they have almost gone away completely.
- Ben had about 17 scalp lesions, ranging from flat red disks to raised yellow waxy papuals with a red base. They are about dime size.
- We are concerned about the 'new' kidney lesion and other new lesions in the brain & lung.
- Ben is doing well - he seems to be developing normally.
- Ben really shows no outward symptoms other than the original eye swelling & seizures and the current scalp lesions - lack of outward or behavioral symptoms does not necessarily imply a positive or good outcome. We have to wait and see.
- Phenobarbital is controlling his seizures and sight loss - no seizures since the medication.
- Because Ben is young, has multisystem involvement and has brain involvement, we are extremely concerned about his long term health and well being.
- Ben has a great disposition and is almost always happy.
- His blood counts have been low at times from the chemo, but overall he has done well.
Prognosis
- Ben has finished 10 weeks of treatment with Prednisone, Vinblastine & Methotrexate (all chemotherapies).
- Ben has also completed 12 courses of 2-CdA (5 days a week about every three or four weeks).
- Ben is just completing 6 courses of high dose Methotrexate (4 or 5 days in the hospital about every three or four weeks)
- We have seen the top doctors for these diseases - Dr. Arceci of Johns Hopkins and Dr. McClain of Texas Children's Hospital and they are consulting on our case.
- We have spent over 75 nights in the hospital and made about 150 trips to the clinic and hospital.
- Ben has been poked over 30 times (he has a porta-catheder).
- Ben has been going to the hospital over 85% of his life.
- We have had 14 MRI scans, 12 CT scans, many body x-rays, and ultrasound and many other tests.
- Ben has had 6 surgeries (eye orbit surgery, hip lymph node & scalp lesion biopsy, brain lesion biopsy, surgery to implant porta-catheter and bone marrow biopsy plus surgery to remove the port and then another to re-implant it).
- For many months we gave Ben several medications orally three times a day.
- We do not know, nor does anyone else know, how well Ben will do and what his prognosis is.
- Benjamin could be on chemotherapy for years.
- Brain surgery would be difficult, if not impossible, for many of the lesions and would only be done in case of emergency.
- Radiation therapy of the brain lesions would damage his young brain and will probably never be done, even in case of emergency.