Lindsey's Journey to a Stem Cell Transplant

Rare disease is often associated with a sense of isolation. Patients feel alone with their suffering, cut off from the healthcare community that may be able to help. We hope to flip the script and make this a story of empowerment, hope, and renewal. You can be a part of this story.

Lindsey (Mattingly) Hughes has battled with autoimmune disease symptoms for years, including interstitial lung disease (ILD), Raynaud’s phenomenon, skin fibrosis, and gastrointestinal issues. However, a single, unifying cause of the disease was not revealed. But in October 2022, an abnormal finding on a colonoscopy/endoscopy led to an official diagnosis of the rare autoimmune disease of Diffuse Systemic Scleroderma (dSsc).

Systemic scleroderma is a multisystemic, chronic disease, in which the body produces abnormal amounts of collagen that lead to the hardening and thickening of the skin, blood vessels, and internal organs. As is true with many autoimmune diseases, the severity of scleroderma can vary from patient to patient, ranging from mild to life-threatening. Unfortunately, Lindsey has encountered a severe and progressive form of scleroderma which has rapidly escalated across her organs. As with many rare diseases, there is no cure for scleroderma; the primary goal is to slow the progression of the disease.

In terms of disease management, Lindsey has worked hard to mitigate the damage that has already occurred before diagnosis. However, she is fighting an uphill battle. Currently, Lindsey requires constant supplemental oxygen to support her declining lung function. Her ongoing drug therapies are causing intense, systemic side effects in addition to the emotional and physical exhaustion of dealing with the disease, and her body has been resistant to standard care medications. Regrettably, Lindsey’s condition has limited her time working at Beechwood High School, which has served as her place of work, refuge, and passion over the past few years. Due to the side effects of her treatment and loss of daily functionality, Lindsey has been on medical leave since January but longs to return to the classroom as soon as possible.

However in the classic, determined Lindsey fashion, she has been on the prowl searching for treatment options around the country. Recently she was able to identify an Ssc clinic at the University of Michigan and is participating in clinical studies which will help benefit other Scleroderma warriors in the future.

In March, Lindsey will enter a critical phase of her disease journey as she undergoes a procedure called Hematopoietic Stem Cell Transplant (HSCT). In essence, HSCT is a “hard reboot” of her malfunctioning immune system. She’ll brave a severe regimen of chemotherapy and irradiation to eliminate malfunctioning immune cells, harvest her own body's stem cells, and transplant them back into her body to reset her immune function. While this is not a cure, this procedure has shown promise in slowing disease progression so that Lindsey can lead a normal, functional life and watch her daughter Ava grow up.

Ava is a very special little one-year-old girl who needs her mommy for singing, playing, and hugs/kisses. As her disease progressed, Lindsey lost the ability to care for Ava on her own, which has been devastating. Lindsey has been blessed with a huge support system who have all stepped in to help. Her husband John has taken on the Herculean role of working daddy, husband and primary caretaker for Ava and Lindsey. However, we need more help.

The HSCT procedure costs $250,000 and will require Lindsey to live with a caregiver at the clinical site for a minimum of 100 days and up to 6 months. Sadly, Lindsey will not be able to have contact with her loved ones, as her immune system will be compromised from the procedure. She will be away from her daughter for this extended period. Lindsey, her family, and her friends humbly ask for your prayers and generous donations to help with some of the financial burdens of medical bills and work leave.

We appreciate any offering of support you could make at this time to help support Lindsey's Journey to a Stem Cell Transplant! We are grateful for all of our friends and family who can assist, and Baby Ava sends love and hugs to all who are cheering on and supporting her mommy at this time!

With love and gratitude,

The Mattingly, Hughes, and Hopkins Families