Living with Spinal Muscular Atrophy: Maylan’s Story (Part I)
Anyone who knows me well knows I have a special place in my heart for a truly remarkable camp known as VACC Camp. VACC Camp is a free week-long overnight camp hosted by Nicklaus Children’s Hospital in Miami for ventilation assisted children and their families. Campers come from all over the United States and internationally for what ends up being their best week of the year.
South Florida families and youngsters from local residential care facilities join in as day campers. The unique camp is a life-changing experience for not only the special needs campers but the many young volunteers who give up their Spring Break in South Florida to be of service to the campers. One such camper was Maylan.
I met Maylan when I first documented the camp back in 2011 (the camp’s 25th year). She was the one that all the volunteers seemed to congregate around. She was beautiful, wicked smart, wicked funny, and she handled her disability with a grace that most of us could never comprehend – much less ever possess. Her disability? Spinal Muscular Atrophy (SMA).
Spinal Muscular Atrophy (SMA) is a genetic disease affecting the central nervous system, peripheral nervous system, and voluntary muscle movement (skeletal muscle). SMA is caused by a mutation in the survival motor neuron gene 1 (SMN1). In a healthy person, this gene produces a protein that is critical to the function of the nerves that control our muscles. Individuals with SMA don’t produce survival motor neuron (SMN) protein at high enough levels. Without this protein, those nerve cells cannot properly function and eventually die, leading to debilitating and sometimes fatal muscle weakness.
SMA affects approximately 1 in 11,000 births in the U.S., and about 1 in every 50 Americans is a genetic carrier. Individuals with SMA have difficulty performing the basic functions of life, like breathing and swallowing. However, SMA does not affect a person’s ability to think, learn, and build relationships with others. Anyone who spends a few minutes speaking with Maylan will hold that truth to be unreservedly self-evident.
Maylan & I, VACC Camp (2016)
I’ve returned to VACC Camp as a volunteer (along with my daughter) every year since my first visit in 2011 and I’ve come to know Maylan better during that time (she aged out of VACC Camp in 2016). I’ve also gotten to see her speak up on social media about her condition and the challenges she has to face as she lives with SMA. One particular series of Facebook posts last year titled “Living with SMA means:” is what gave me the idea to partner with her to create a series of photographs (along with her words) that will hopefully help further outline what living with SMA means.
And being that August is also SMA Awareness Month, we agreed that this was the month to share her story.
After agreeing to a date, I arrived at Maylan’s apartment in Miami around 11am (like myself, she’s not a morning person) and she was already up in her adjustable bed, phone in hand. I’ll let the photographs (and Maylan) take it from here…
Individuals with SMA have very weak intercostal muscles (the muscles between the ribs), which compromises normal breathing rhythm. In Maylan’s case, this led to the need for breathing support in the form of a tracheostomy. A tracheostomy is often needed when health problems require long-term use of a machine (ventilator) to help you breathe.
Maylan: “It’s a bittersweet thing. You go through the motions. You fear looking “more disabled,” you’re made uncomfortable by having to lug around all this equipment, you feel less approachable and alienated because people find you intimidating or scary. But nonetheless…you grow to love it. Because it’s the very thing breathing life into your lungs, the very reason why you’re still alive to keep experiencing all of this world’s wonders.”
Maylan’s SMA has progressed to the point where she has to rely on someone to help her with most, if not all, of her daily activities. That person is her remarkable nurse Ruth, who spends 16 hours a day with her several days a week.
Maylan: “Because of the nature of the care I receive, the bond between me and my nurse must be founded on trust and understanding. The trust that my life is in her hands, and the understanding that she is here to help me live my life like anybody else. We chat and we joke around. We cry and have happy moments. We work together and communicate to achieve many things throughout the day together.”
I step out of the room as Ruth gets Maylan ready for her morning shower. When I’m called back in, Ruth is performing tracheostomy suctioning, which removes thick mucus and secretions from the trachea and lower airway that Maylan is not able to clear by coughing. This is a morning ritual.
Maylan: “Showering as a disabled person is a lot of things. It is refreshing, uncomfortable, intimate, vulnerable, physical, and taxing. It is one of the many things taken for granted as living with a disability can sometimes mean having no accessible bathroom, have no suitable equipment to transfer or sit in the bathroom, and/or not having the adequate help to get into the shower.”
Maylan: “Showering is perhaps the most physically taxing part of my day as I need to be carried out of bed, into the chair, back in bed, and later, back into my wheelchair. Toileting is also difficult. In my experience, it is the most challenging aspect of my daily life. So most definitely blessing number infinity is having a commode chair and a superhero nurse to help me freshen up.”
I step out again so that Ruth can dress Maylan, who takes how she looks quite seriously…
Maylan: “Being the sublime fashion goddess that I am, I always enjoy looking fabulous. But since it’s a casual day, I throw on a nice little dress.”
Maylan: “After picking out an outfit, Ruth disconnects me from my ventilator and carries me into my throne, my beloved wheelchair that gives me the independence to move around.”
Maylan: “After being sat, we make a few more outfit and seating adjustments and transfer my ventilator onto the back of my wheelchair. All the while I simply consider myself so lucky that my nurse understands the nuances and finer details of my comfort and adjusts me accordingly.”
Maylan: “I have a nice handy dandy lightweight toothbrush, but even that is heavy for me at times which makes it difficult to brush my teeth on my own. Today, however, I feel enough strength to do it.”
Maylan: “Being a young and spicy Latina who’s very proud of her curls, and yet unable do her hair by herself, the grooming part of every day can be a difficult process. I wish I could do my hair on my own sometimes. I wish I could have the ease of raising my hands over my head and simply moistening my hair, applying product, then styling it myself. Nonetheless, where SMA taketh, SMA giveth. And I’m happy I have a nurse like Ruth who won’t ever let me leave the bathroom without looking flawless.”
Maylan: “Eyebrows, eyeshadow, a little mascara, light hint of foundation, and the most important part, my signature – a bright, bold lip! The one thing I can do is my lipstick. And have you no doubt that I make sure my lipstick always looks immaculate. I don’t ever let anybody else do it for me.”
Maylan: “A lot of times, people with the best of intentions will approach me and treat me as if me being out and about (and looking beautiful) is some sort of novelty to gawk at when in reality, they couldn’t be farther from the truth. They perpetuate the stigma that just because I am disabled, I must presumptuously look sickly or lackluster. But boy, I am here to tell ya that in all my disabled goodness, I can still enjoy and live out the pleasures of dressing up and looking cute.”
Maylan: “I take advantage of the little bit of time I have while Ruth continues to get ready to play one of my favorite games, Animal Crossing. I love to game, but much like brushing my teeth, sometimes it requires a certain level of strength and dexterity that some days I simply don’t have. I feel that gaming has been such an enjoyable part of my life and the fact that SMA has slowly taken my ability to do it at times saddens me. Let us advocate for increased inclusion and accessibility in gaming so that all may participate in it.”
In the kitchen, a photograph of a young Maylan adorns the refrigerator. Maylan, who was born in Cuba, was diagnosed with SMA when she was barely a year young. Doctors in Cuba were unable to properly diagnose her and told her parents she would most likely die before the age of two. However, when Maylan was 16 months old, her parents were fortunate enough to win the visa lottery which grants visas to the United States to only a few thousand people worldwide each year.
Maylan: “Life as I knew it was turned upside down by the Coronavirus. Its made everything feel unsafe. Having SMA means that we’re immunocompromised due to our weakened respiratory system, so the prospect of catching something as deadly as the Coronavirus is horrifying at the least. I wish all of us would realize that this pandemic affects everyone. Anyone can have a disabled friend, family member, acquaintance, or partner so it’s our collective duty to take care of one another and mask up.”
Maylan: “We head out the apartment building to my beloved, Vanny Devito (my van). Vanny is another blessing in my life that many take for granted: having transportation. A van with accessibility for a wheelchair are hard to find. They’re oftentimes expensive, scarce, and just generally unobtainable to many disabled people. I was able to buy one thanks to the amazing help of my friends and family, but without them, I wouldn’t have been able to have this tool of independence and freedom in my life.”
After a quick run to pick up lunch at the local KFC drive-through, we return to Maylan’s…
Maylan: “Eating is yet another thing that I value greatly because I’m still able to eat by mouth and in most cases, still able to feed myself. But living with SMA means you oftentimes need help for everything, and this naturally includes eating. Sometimes I get too tired to feed myself or even too tired to eat at all, even if somebody else feeds me.”
Maylan: “After our good lunch, I go back to my room to suction again and relieve a little bit of breathlessness from the day’s activities.”
Maylan: “Then I get on my Eyegaze, my wonderful piece of assistive technology. The Eyegaze is a tablet which allows the user to use the computer hands-free through eye tracking technology. It is for people with limited mobility, like myself, to have access to communication, socialization, and all the functions of a regular computer. Like many of the things that make my life easier, however, the Eyegaze is also expensive and difficult to obtain by normal means. We need to fight and advocate together for assistive technology and everything in between to be easier to acquire so that people with disabilities can be included and integrated in life as we deserve.”
EPILOGUE
Maylan and I hope you enjoyed Part I of our series. Our goal is that it will create awareness and understanding for those living with SMA – and maybe even inspire someone along the way. For a young girl who was not supposed to see her second birthday, Maylan will turn 27 next month – she’s defied the odds. And in the process, has impacted the lives of so many. Me being one of them.
This is Part I of my series, stay tuned for Part II later this week. For more information on SMA, online communities and advancements in treatment, please visit www.curesma.org
