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Madeline Holt’s story is one of hope, love, and community.

Madeline Holt’s story is one of hope, love, and community.

Madeline Holt’s story is One of Hope, Love, and Community. Madeline Holt’s story is one of hope, love, and community. Maddie was born in November of 2012 at 28 weeks gestation. She weighed 2lbs 3 ounces at birth and spent the first three months of her life in the NICU. Her care team was proud of her continued improvement and development, congratulating her parents on their miracle baby. Maddie, now weighing 5 pounds, was discharged from the hospital with formula, a prescription for omeprazole to treat her gastric reflux, and hope that despite her premature birth and mild hearing loss, she would continue to progress and thrive with the love of her parents, Meagan and Brandon Holt.

“I am doing what I have to do. Would you just give up if your kid was born with a terminal illness? You can’t be okay with that.” ~ Meagan Holt (Mother of Maddie)

Things weren’t so easy for Maddie and her family after her discharge from the hospital. As the Holts began adjusting to their life at home with Maddie, they noticed that her hearing loss was more severe than was initially thought. Maddie didn’t respond with a startle stimulus when a door slammed or when their small dog barked near her.

They also noticed that she didn’t visually track items in the middle of her visual field. Maddie was bottle fed and still needed to eat every few hours. Her suckling rhythm was unique, and not very efficient. Her mother had to finesse the way that she bottle fed Maddie, attempting to get enough nutrition into her. When Maddie started projectile vomiting after small feeds, her family and care team decided that she would have a gastric feeding tube placed to ensure adequate intake of nutrition and fluids. During these first few months at home, it seemed like a new symptom and diagnosis happened every couple of weeks, along with new consultations and many follow up appointments.

Maddie receives her care at Seattle Children’s Hospital. One of her care teams is the Craniofacial team. The team observed that Maddie had some key facial features that were similar to her fathers. Also, one of the fontanels in her skull was still 6 inches across, a huge open head space. This led to a consultation with the Biochemical Genetics team during the summer of 2013. Biochemical genetics involves diagnosing and treating metabolic diseases. Blood was drawn from Maddie and sent it off to Johns Hopkins Hospital for DNA sequencing and evaluation. The results came back on October 30, 2013, two days before Maddie’s first birthday.

Maddie has Zellweger Syndrome, a terminal genetic disease that destroys the white matter of the brain. It is the most severe form of a group of four related diseases called Peroxisome Biogenesis Disorders (PBD). Peroxisomes are cell structures that break down toxic substances and synthesize lipids (fatty acids. oils, and waxes) that are necessary for cell function. Peroxisomes are required for normal brain development and function and the formation of myelin, the whitish substance that coats nerve fibers. They are also required for normal eye, liver, kidney, and bone functions.

Zellweger Spectrum Disorders result from dysfunctional lipid metabolism, including the over-accumulation of very long-chain fatty acids and phytanic acid, and defects of bile acids and plasmalogens–specialized lipids found in cell membranes and myelin sheaths of nerve fibers. Symptoms of these disorders include an enlarged liver; characteristic facial features such as a high forehead, underdeveloped eyebrow ridges, and wide-set eyes; and neurological abnormalities such as cognitive impairment and seizures. Infants with Zellweger Syndrome also lack muscle tone, sometimes to the point of being unable to move, and may not be able to suck or swallow. Some babies will be born with glaucoma, retinal degeneration, and impaired hearing. Jaundice and gastrointestinal bleeding also may occur.1 Zellweger Syndrome is caused by a mutation in any one of 12 genes and is inherited in an autosomal recessive manner, which means both parents carry copies of the mutated gene.

Maddie had her first grand mal seizure in January of 2015. Before this Maddie could sit, roll on a flat surface, knew 100 signs that she could communicate with, and her only medical interventions were a feeding tube and omeprazole for gastric reflux. Daily seizures changed everything. Maddie’s team tried 26 different pharmaceutical medications trying to control her seizure activity and to keep her comfortable. Her personality and interaction with others were severely blunted by her medications, their side effects, and ongoing grand mal seizure activity. More medications equaled less Maddie. She was oxygen dependent, very sensitive to narcotics, and required CPR numerous times at home and in the hospital.

April of 2015 was a difficult month for the Holts and their care team at Children’s Hospital. Brandon and Meagan were told that the medical team was out of options, having nothing left for treating Maddie’s disease and that it was time to make an end of life plans, due to her medical fragility and impending death from seizures. Maddie was enrolled in Hospice, adopted DNR status, and was prescribed morphine for her comfort.

The Holts were not ready to accept this. Meagan googled pediatric intractable epilepsy. Her search results included Landon Riddle, projectCBD.org, and HaleighsHope. She did more research, finding that cannabis had improved quality of life for many children with life-threatening diseases, but Maddie didn’t fit into any one group: deaf, blind, deaf/blind, cancer, or epilepsy. Seeing that there was no road map for Maddie’s care, Meagan and Brandon decided they had nothing to lose and everything to gain. They pursued alternative care using cannabis. They were fortunate to live in Washington State, which had a strong medical marijuana law at the time.

Maddie started using Full Extract Cannabis Oil (FECO) containing CBD under the care of Dr. Katrina Iiams-Hauser, hoping for an improvement in her physical and mental comfort and reporting her use and its effects to her care team at Hospice and Seattle Children’s Hospital. Refinement of her treatment regimen included adding THC. Maddie responded with an increased expression of emotion, increasing motor skills, and re-learning sign language.

Community and Canna Family are essential to Maddie’s ongoing use of cannabis. Insurance does not cover the cost of cannabis products, even when authorized by a medical professional. Donations of pesticide-free and lab tested FECO come from medical growers, processors, and members of the medical marijuana community in Washington State. Maddie not only lived but has thrived on her cannabis regimen. Two years later at age 4, she is no longer in hospice, has stopped having grand mal seizures, and continues to improve physically and cognitively. She will always be a medically complex patient.

Cannabis did not cure her, but enables her to participate in life and decreases her suffering. She is homeschooled one day a week, and her progress awes and inspires her teachers, parents, and caregivers.

“We watched her do everything she never was supposed to do” Meagan proudly states.

Maddie loves music and socializing. Her palliative care team has weaned her pharmaceutical use from a high of 26 drugs in April of 2015 to just 5 in addition to cannabis products. Her current medication list includes: hydrocortisone, Keppra, clonidine, vitamin K, and a aquADEKS (a fat-soluble multivitamin), 45mg of CBD & 20 mg of THC twice daily in the form of FECO, 10 mg THCA twice daily, and as needed for stress, a 5 mg THCa isolate as needed for seizure rescue, among other therapies. https://youtu.be/OrLyA-NZpfY (9:30), as well as topical applications to control symptoms caused by Zellweger syndrome. Maddie spent more than 200 days at Children’s Hospital in 2015, about 30 days in 2016, and a week in 2017 for treatment of Rhinovirus.

Zellweger syndrome

Zellweger syndrome

The Holts have always been open with their care teams about Maddie’s use of cannabis for the quality of life. “I give her a plant. I NEVER have and never will hide Madeline’s cannabis medicine from her providers because it is crucial to her care. I am completely open and honest about dosing, administration, cannabinoid content.

Through positive engagement, by reminding her providers that I am a critical member of her care team they listen. Imagine being in their shoes. Imagine telling a family; it’s time to start making an end of life plans; we are out of options. Imagine that same child coming back to the hospital off of all those drugs you knew she would die for? Imagine watching cannabis save a life?

I advocate for cannabis patients and the providers who are desperate for research. By educating my daughter’s medical team, showing them cannabis works, they support us. They uplift us, and they accept cannabis as a part of her care even when being admitted”. Children’s Hospital does not administer or provide any cannabis products to Maddie but does not obstruct the Holts from administering the cannabis products to Maddie while she is an inpatient.

Holt also credits Children’s Hospital for helping her develop her advocacy skills and giving her a more powerful voice through the supportive programs they offer to patients and families.

Meagan has been a strong and vocal advocate of families affected by rare diseases, patient rights, for cannabis education, and safe access to cannabis. She has worked with groups such as Hempfest, NORML Women of Washington, 22 Too Many, and Project PC. She publicly protested the profound changes made to the medical marijuana law in Washington State by House Bill 5052 in 2016 (ironically titled The Patient Protection Act 2 ).

Maddie engaging with Pediatric Nurse Juhlzie with mother, Meagan Holt. April 28, 2017

Maddie engaging with Pediatric Nurse Juhlzie with mother, Meagan Holt. April 28, 2017

Schools have the power to adopt cannabis policies for children who need it in Washington State. However, no school currently has a policy for children to use cannabis at school. That leaves special needs students like Maddie, who use medical marijuana in non-smokeable forms throughout the day, without access to safe education.

Meagan initiated legislation in 2017 to allow non-smokeable administration of medical marijuana on school grounds for pediatric medical marijuana patients through her legislative representative. She testified at the hearing for House Bill 1060, also known as “Maddie’s Law” and “Ducky’s Bill,” sharing Maddie’s life story with the committee members with the hope that the bill may allow Madeline to attend kindergarten. The bill remains in committee for the 2017-18 legislative session, meaning Maddie will be homeschooled for the next year.

What’s next for the Holts? Along wished-for family trip to Disneyland, ongoing legislative and educational efforts to normalize and ensure safe access to cannabis for children at home and school, and the publication of Meagan’s poetry and journal entries, titled Just Breathe Baby. Meagan will be speaking at Hempfest in Seattle (August 18, 19, &20, 2017). Maddie’s progress can be followed at Madeline’s Whole Plant Journey on Facebook.

 

Resources:

https://www.ninds.nih.gov/Disorders/All-Disorders/Zellweger-Syndrome-Information-Pag

Hundreds of medical marijuana shops to shut down with new state rule kicking in Friday

Other Resources/Videos on Maddie:
https://www.ninds.nih.gov/Disorders/All-Disorders/Zellweger-Syndrome-Information-Page

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