Patient Story: A Young Boy Finds Help at PNI for Pediatric Cushing’s Disease
by Guest Author
A devoted father searching around the world for help finds the care his son needs at Pacific Neuroscience Institute. Read Basab’s story below.
A Search for Medical Help and Hope
One can only imagine the anguish of Deg Gharti Chhetri by the fall of 2018. Deg had journeyed around the world—Singapore, Thailand, India and the United States—with his young son, Basab Gharti Chhetri, from their home in Kathmandu, Nepal, in search of medical help for his child. But Basab was still suffering from Cushing’s disease—caused by a benign tumor in his pituitary gland—after almost three years of treatments.
“No young child should go through this,” says Deg of the many tests and treatments Basab received.
Ultimately, late last year while staying with a nephew in Atlanta, Deg sent an email to Pacific Neuroscience Institute (PNI) at Providence Saint John’s Health Center. He was intrigued by information on a website detailing PNI’s expertise in the treatment of all kinds of brain and pituitary tumors, including those that cause Cushing’s disease. Deg didn’t know it then, but he had finally hit upon the right combination of skill, experience and compassion that would restore his boy to health.
“It started with an email from Deg,” says neurosurgeon Daniel F. Kelly, MD, director of PNI and director of the Brain Tumor Center and Pituitary Disorders Center. “He laid out for me what had happened and how distraught he was, and he asked whether another surgery was an option and what we would recommend. This man had spent a fortune in treatments for Basab, and he was unfortunately getting sicker.”
Basab is just the kind of patient that surgeons at PNI welcome. In addition to performing a broad range of procedures on common malignant and benign brain tumors and pituitary tumors, Dr. Kelly and his colleagues are comfortable with unusual and complex cases or ones that have befuddled other neurosurgeons and neurologists. In contrast to medical centers that might see a few cases each year of pituitary tumors including those with Cushing’s disease, the PNI team treats approximately 100 cases per year.
“The outcomes are generally quite different at pituitary centers that do a consistent high volume of such pituitary surgery cases,” Dr. Kelly says. “If you go to an experienced center, your likelihood of getting done right the first time is higher. There are circumstances where we don’t cure patients because of the biology or invasiveness of a tumor, but the remission rates in experienced hands are generally excellent.”
Removing the tumor through the nostrils
Cushing’s disease occurs when the paired adrenal glands (which sit atop the kidneys) produce too much of the stress hormone cortisol that is involved in maintaining blood pressure and blood sugar and turning food into energy. Although the adrenal glands are producing too much cortisol, the origin of the disease, as in Basab’s case, is a benign tumor (adenoma) of the pituitary gland, which is the master gland of the body, orchestrating all hormonal functions, situated directly below the brain in the base of the skull. The small tumor produced too much of adrenocorticotropic hormone (ACTH), which causes the adrenal glands to produce too much cortisol.
Cushing’s disease can be difficult to diagnose in children and adults. Basab’s uncle, who is a surgeon, noticed the child’s puffy face and short stature (both telltale symptoms) in 2016 and urged the family to take him to an endocrinologist. That summer Basab was diagnosed with Cushing’s disease.
But the family’s odyssey was just beginning. They visited doctors in Nepal and India before they chose a surgeon in Singapore to remove the pituitary tumor. However, the seven-hour surgery failed. Not enough of the tumor was removed, and Basab’s cortisol levels remained high. “They got a lot of the tumor out, but you have to get it all out to get people into remission,” Dr. Kelly explains.
Still searching, Deg learned about a surgical procedure to remove the tumor with Gamma Knife radiosurgery, which uses highly focused radiation beams to destroy tumors without damaging healthy surrounding tissue. Deg found a surgeon in Pittsburgh, and in May 2017 the family traveled there for the radiation treatment.
The Gamma Knife radiation however also failed. One year later an MRI scan showed the tumor was still present. Medication to lower cortisol levels also failed to help the child and his symptoms of weight gain and arrested growth persisted. That’s when Deg found Dr. Kelly after considering another surgery at Harvard, Children’s Hospital of Philadelphia, Johns Hopkins Hospital, the Mayo Clinic and other centers.
After many phone calls and emails between Deg and Dr. Kelly, the family flew to Los Angeles—a 16-hour plane ride covering 8,000 miles. On March 5, Basab underwent surgery at Providence Tarzana Medical Center, where there is a pediatric unit—reflecting the type of collaborative spirit among sister hospitals that the Providence St. Joseph Health system is known for.
“When we met them, I felt like I knew them,” Dr. Kelly says. “Deg had sent me pictures of Basab. They are the nicest, most humble people. Basab never complained. For most 10-year-olds, to do an MRI you have to sedate them. He went right in. He never cried about IVs. I’ve never seen such a stoic but cheerful kid. People just fell in love with him.”
Dr. Kelly and Chester F. Griffiths, MD, director of the Eye, Ear & Skull Base Center at PNI and an ENT and facial plastic surgeon, performed a three-hour procedure to remove the tumor, inserting instruments through the nose and sphenoid sinus of the skull base up to the pituitary gland. The approach, called endonasal endoscopic surgery, is a team surgery that is now used for the vast majority of pituitary tumors, tumors of the skull base as well as some midline brain tumors.
The surgeons use a high-definition, 4-millimeter endoscope to provide a highly magnified and detailed panoramic view through one nostril while there are instruments working through both nostrils. Most patients are in the hospital for a night or two and can return to full activities within three weeks of surgery.
“We have a beautiful view of the surgical anatomy,” Dr. Kelly says. “Even in young kids like this, a small 10-year-old, we can remove such tumors through the nostrils without any incisions on the face. It’s a truly minimally invasive approach. We take a lot of different tumors out with this approach: pituitary adenomas, meningiomas, chordomas, Rathke’s cleft cysts and craniopharyngiomas.”
Getting well and growing up
Within hours of surgery, Basab’s cortisol and ACTH levels dropped into the low (non-measurable) range, indicating that the tumor was completely removed. Since then, his levels have stayed low and he is on cortisol replacement with a medication called hydrocortisone, prescribed by Dr. Norman Lavin, a pediatric endocrinologist at Providence Tarzana Medical Center who collaborates with Dr. Kelly and Dr. Griffiths.
Basab will likely need the hydrocortisone for up to a year while his pituitary gland recovers and the adrenal glands get their normal biological rhythm back. After surgery, he has lost weight caused by the excess hormone levels and is again growing.
“My young Basab loves to enjoy watching soccer during his free time,” Deg says. “His favorite player is Cristiano Ronaldo.”
“He is doing well,” Dr. Kelly says. “What happens with Cushing’s is the cortisol and ACTH levels need to bottom out immediately after surgery. Then you have to give them the steroid back. He should start beginning to grow again and ultimately lead a normal life.”
Basab’s case is a good example of why it’s important for patients with challenging conditions to seek care at an experienced center or a “center of excellence,” says Dr. Kelly, who has one of the world’s largest endonasal pituitary surgery experiences with more than 2,000 surgeries performed and more than 60 peer-reviewed publications relating to endonasal surgery for pituitary tumors.
The family returned to Saint John’s in October for a follow-up visit. It is a chance for a child, a family and a team of healthcare professionals to appreciate health and happiness.
“Basab is happy,” Deg says. “He has something to live for and to be happy about. He is looking forward to starting school again and being with his friends. I would like to thank Dr. Kelly for everything because no one else would have done this for my child.”
Watch Basab’s story which was featured on NBC Los Angeles.
Pacific Pituitary Disorders Center
We provide personalized, comprehensive care and endoscopic surgery for patients with pituitary tumors and related hormonal health and vision problems. Contact the Pacific Pituitary Disorders Center to schedule a consultation, record review, or second opinion.
Written by Shari Roan and featured in Saint John’s Health Center Breakthroughs magazine, Fall 2019
Banner image: Dr. Daniel Kelly, Basab and Dr. Chester Griffiths
Last updated: September 10th, 2020