Meet Harper, who passed on from CDKL5

/ / Featured, ECHO Families

Harper Howard | CDKL5

April 12, 2016 would’ve been Harper’s 6th birthday, and although we are unbelievably sad that she is still not here on this Earth with us, we are happy to share with you the amazing influence this little girl continues to have on the world around us. Harper Howard was born with a rare disease known as CDKL5, a non-hereditary neurological disorder characterized by frequent seizures. This is her beautiful story:

Harper had her first seizure at just two weeks old and from that point on, began suffering from up to 40 seizures a day. Epilepsy is an incredibly horrendous disease – Every time the body seizes is a gamble with life. The strain of these seizures stole Harper’s personality from her, leaving her physically and mentally drained. Traditional pharmaceuticals were ineffective at preventing these seizures and at times left Harper “catatonic”, according to Harper’s mother, Penny.

With less than 600 cases worldwide, Penny began looking to raise awareness of this uncommon disease and founded the Hope4Harper Foundation to act as a support group for other CDKL5 families. There, she began to blog about her daughter’s disease – a blog that would become very important in inspiring a countrywide movement in Brazil.

Soon they came upon Sanjay Gupta’s CNN special, Weed, where they discovered Real Scientific Hemp Oil™ – a CBD oil derived from hemp. They continued their research and found countless articles about how CBD, a cannabinoid found in cannabis, was helping treat epilepsy.

Harper’s parents, distraught over over their daughter’s ineffective medication cocktail began searching for an alternative. Soon they came upon Sanjay Gupta’s CNN special, Weed, where they discovered Real Scientific Hemp Oil™ – a CBD oil derived from hemp. They continued their research and found countless articles about how CBD, a cannabinoid found in cannabis, was helping treat epilepsy. Cannabis wasn’t legal in their state of Texas, but since RSHO™ was derived from hemp, it was legal for them to try with their daughter!

After integrating CBD hemp oil into Harper’s strict dietary regimen, her seizures began to diminish: first from dozens a day down to 3-4, then to none. At one point it was even able to eliminate Harper’s seizures for 45 straight days. As incredible of an accomplishment this was, this wasn’t even the best part! According to Dustin, after adding CBD hemp oil, Harper’s “personality began to come out”, including visual and verbal interactions and even laughter.

After the success the Howards experienced using RSHO™, Penny shared their story on the Hope4Harper blog and on the foundation’s Facebook page. Not long after, another mother of a CDKL5 daughter was searching for answers of her own 5,000 miles away in Brazil. After seeing the improvement being documented by Harper’s parents, Katiele Fischer decided to try CBD to treat her daughter, Anny and began illegally importing RSHO™ into Brazil with the help of family they had in the United States.

Within 9 weeks, Anny enjoyed her first seizure free day. Unfortunately, not long after, the Brazilian Customs Department seized Anny’s next shipment, preventing her from continuing treatment. Acting fast, the Fischers took their case to the Brazilian Courts, and in just three days, the government declared it “inhumane” to keep RSHO™ from Anny. News of Anny’s victory would eventually lead to the legalization of CBD across Brazil as a prescription medication for nine separate indications.

A similar series of events unfolded in Mexico. Grace Elizalde suffered from up to 400 seizures a day after being diagnosed with Lennox-Gastaut syndrome. After research brought Grace’s father, Raul, to find CBD hemp oil, he petitioned the Mexican government to allow CBD products to be imported into the country. Hoping to spread the word on the success they’d had with this pharmaceutical alternative, Grace’s father started the Por Grace Por Todos Foundation, which helped lead to the legalization of CBD in this country of 120 million.

In the meantime, while little Harper quietly affected change across the globe, she continued to grow and was able to more fully live her life with the help of CBD. 

In the meantime, while little Harper quietly affected change across the globe, she continued to grow and was able to more fully live her life with the help of CBD. She would play with her brother and sister and go horseback riding each week, and in 2015, she was all smiles as she was crowned Miss Texas Princess Cover Girl at the National American Miss Pageant.

She was a vibrant, inspiring, beautiful little girl, but unfortunately on January 8, 2016, Harper’s disease prevailed and took her from this Earth. Although Penny and Dustin knew this day would come, it was still incredibly difficult to lose this light in their life, but they had discovered a way to continue letting Harper inspire change far beyond her worldly existence – Harper’s body would be donated to science!

…they had discovered a way to continue letting Harper inspire change far beyond her worldly existence – Harper’s body would be donated to science! 

Last week, on what would have been Harper’s 6th birthday, Penny and Dustin flew into San Diego to see how their daughter’s cells were progressing local scientists’ research on neurological disorders at UC San Diego. What they witnessed was heart warming – There, in the petri dish they held in their hands, were their daughter’s cells alive and well. Because of Harper, scientists will now have the opportunity to truly research the effect of CBD (cannabidiol) on the human brain.

This research is being lead by Dr. Alysson Muotri, head of the Muotri Lab at UCSD’s Rady Children’s Hospital. Dr. Muotri earned his Ph.D. in Genetics from the University of Sao Paulo before moving to the Salk Institute in La Jolla to complete his postdoctoral training in neuroscience and stem cell biology. He has received over two dozen honors and awards, including the NIH Director’s New Innovator Award and the EUREKA NIMH Award for “exceptionally innovative research”. Now, with his own lab within the UCSD Department of Pediatrics, Dr. Muotri is focusing on human brain development and evolution, using stem cells to investigate human neurological disorders.

In discussing the importance of the donation of Harper’s tissue, Dr. Muotri points out the significance of having brain and skin cells from Harper in their research. Rather than the typical donation of post-mortem cells, which are not as effective for research, Harper’s stem cells can be used to create “mini-brains” in the lab to better research the effects of a number of possible medications, including CBD, on a CDKL5 brain.

Rather than the typical donation of post-mortem cells, which are not as effective for research, Harper’s stem cells can be used to create “mini-brains” in the lab to better research the effects of a number of possible medications, including CBD, on a CDKL5 brain.

According to Dr. Muotri, this will “speed up the process of drug discovery, and perhaps we can go into clinical trials much earlier than if we used previous models.” He goes on to add that Harper’s donation will be able to be used beyond just research into CDKL5 and into a number of neurological disorders.

Harper’s brain is also unique in that it has been treated for 28 months with CBD hemp oil. By conducting research on a CBD affected brain, Dr. Muotri can better understand the role cannabis plays in the treatment of brain disorders. Dr. Muotri is also “currently testing the effects of hemp oil in these mini-brains to see if it can inhibit seizures.” He goes on to discuss the need to understand the long-term changes that take place in the brain’s neurons. However, preliminary research shows a “decrease in neuronal firing noise”, which can help in controlling seizures.

By conducting research on a CBD affected brain, Dr. Muotri can better understand the role cannabis plays in the treatment of brain disorders. Dr. Muotri is also “currently testing the effects of hemp oil in these mini-brains to see if it can inhibit seizures.”

During their tour of the lab, Dr. Muotri explained the work that has been done to prepare Harper’s cells during research, and Penny was even able to hold the living cells of her daughter again – a beautiful moment for their family.

The Howards see the work being done by Dr. Muotri as an extension of Harper’s legacy of hope. Their family’s forethought and compassion in donating Harper’s cells to research is vitally important to furthering our understanding of this incredibly rare disease. Through this research, little Harper Howard continues to live on, influencing positive change and now scientific discovery!

In speaking about their daughter’s final sacrifice, Dustin opened up to us. “I hope that her final donation to the world will bring something very positive.” Praising the work of Dr. Muotri and his team at UCSD, he continued on to say, “With what we saw today, we are 100% sure it will lead to something huge!”

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