I’ve failed. For months now, I've tried again and again to write this post, only to stop midway through. It's been hard because I just get tied up in knots and can't find the right words for this story.
Meet the Dipo family. Gorgeous, right? I’ve photographed them twice, and I think you’ll agree, they are straight out of a Ralph Lauren ad. Model gorgeous, with bright blond hair and blue eyes — come on! You would never guess that they are going through one of the hardest struggles I have personally ever witnessed. Their baby, Miles, has a rare genetic disease, called tuberous sclerosis complex (TSC). He is extra special in that he has an extremely rare mutation on the TSC2 gene that has riddled his body with benign (non-cancerous) tumors.
This is their story
At 19 weeks, in utero, a tumor was first detected inside his heart that was restricting his growth. The blood was so restricted to his heart that he plunged down to the 7th percentile. This was just the start of multiple doctor appointments and a glimpse in to the frustrations Jenny would encounter. She was informed that her toddler son could not be present at any of the future appointments. Jenny faithfully chronicles their families journey in her exceptional blog, and this is an excerpt from 3/17/2016:
Baby Miles would eventually dip down to the 1st percentile, while the cause of his tumor was speculative. Some doctors viewed only a single cardiac tumor with no other disease association, while the physicians at Seattle Children’s suspected that there were multiple cardiac tumors – a typical early symptom of tuberous sclerosis.
The family went through momentous ups and downs in their struggles. The struggle is between pushing/fighting with all their might for something that is needed— whether it be information, resources, better doctors, or courage and strength — and then in the same breath, turning around and letting go of all control. Watching it is difficult, but when I close my eyes and really try to imagine the pain they must have gone through, I can hardly breathe.
By 35 weeks, Miles (his name means soldier), had outgrown his tumor, allowing blood flow to be restored to his heart. He reached the 10th percentile and was deemed okay for a full-term delivery. It doesn’t stop there though.
May 16, 2016, beautiful Miles Dipo is born! Miles has 8 tumors in his heart. Miles has many tumors in his kidneys. Miles has 7 large tumors and several other suspects in his brain. Miles has tuberous sclerosis.
Miles got to go home and have a brief respite with his family, but essentially in lock down. His fragile health required the family keep visitors to an absolute minimum, while they worked with doctors to try to accelerate the regression of the heart tumor with an immunosuppressant medication in order to try to avoid heart surgery. His treatment had decent research behind it, but was still experimental, and without any assurance the medication would work.
After just over one week at home, it was determined that the heart tumors damaged his aortic valve such that it needed to be replaced. At three weeks old, he underwent a delicate, and extremely complicated open heart surgery, called the Ross Procedure. He was intubated, and therefore sedated, for a full 9 days. He was tube fed, and eventually became dependent on the painkillers. All this while his loving parents stood beside him feeling defenseless to this terrible disease. After an overwhelming ordeal and nearly three weeks, they were cleared to return home with Miles, and were reunited with their two-year-old back on Bainbridge Island. Miles needed around the clock care at this point.
The next challenge they faced was infantile spasms, a condition where TSC is one of the leading causes. They are considered an emergency because children will stop developing normally and lose skills after the spasms begin, so preventing them and identifying them is critical. Sadly, 40% of people with TSC will develop infantile spasms, and Miles’ EEG at birth had shown spikes, indicating he was even more likely to have them. 80-90% of people with TSC will have seizures. Of those, 68% will develop intractable epilepsy. This begins their next journey. Every few months, Miles endures sleepless days and nights while being monitored via EEG for 24 hours at a time, in hospital. When the technician placed the electrodes on his head and exfoliated too hard at Miles’ first outpatient EEG, it caused him to cry so hard that he would pass out momentarily.
Jenny’s fiercely advocated for Miles. She learned of an early study on an anti-seizure medication (vigabatrin) that was tested as a preventative measure for kids with TSC, and that seemed to have rather compelling results. Getting that drug prescribed when it is not yet the standard of care in the United States proved extremely difficult, but Jenny didn’t stop until she found a doctor who was an advocate and likewise believed in a proactive approach, and that the possibility of a much brighter outlook for Miles’ life would offset the potential risks of the drug.
But now for the good news, his most recent 24-hour EEG results were completely normal! To date, Miles is seizure free, and beginning to speak and babble! He recently began crawling. He is extremely social and loves being around his brother. Please keep on fighting, soldier!
And yet, Miles is still not out of the woods. He will likely need at least one or two more heart surgeries during his youth, as well as a few more into adulthood, to accommodate his growing body and aging replacement valves. Although seizures have been prevented so far, they remain a very likely scenario for him at some point, even if they can completely avoid the devastating infantile spasms. The tumors in his kidneys or wherever else they may develop could likewise have negative impacts on him later in life. But for now, he is the joy of the Dipo family, and is doing everything a 10-month-old is supposed to, for which they are tremendously grateful.
Go the Extra Mile
So, what can you do if you’re touched by their story and want to do something to help their family? The Dipo family has a very simple request. Visit their team page for an upcoming fundraising walk, and donate what you can to help raise awareness and critical funding for research into this devastating genetic condition. Plan on joining us for The Step Forward to Cure Tuberous Sclerosis 5K, at Magnuson Park in Seattle on June 24, 2017 at 9am. We will depart on the 7:55 a.m. ferry. Effective treatments continue to be developed, and the disease is believed to be on the brink of a breakthrough.
And for Amazon subscribers, use Amazon Smile to make your purchases. To set this up click here and, select your organization (National Tuberous Sclerosis Association). Now the AmazonSmile Foundation will donate 0.5% of the price of eligible purchases to the charitable organization.
And to close this, I’ll leave the final words to Jenny. This is an excerpt from her 9/1/16 post: