September is Sickle Cell Disease Awareness Month. Sickle cell disease (SCD) is a blood disorder that affects approximately 100,000 Americans, of whom 6-11 percent may develop pulmonary hypertension (PH). The Pulmonary Hypertension Association (PHA) is recognizing SCD Awareness Month by highlighting a prominent PHA volunteer living with SCD-associated PH.

Imagine a six-month-old screaming in pain, inconsolable, with no visible indication of anything physically amiss. It would be determined, after a blood test, that sickle cell disease was the culprit.

Nicole Creech, now 46, has fought and won many hard battles with SCD. As a sickly child, she was teased and mocked by other children her age and told that she would only live to see the age of 15 – if that. SCD is a blood disorder that causes a person’s red blood cells to take on a ‘sickle’ shape rather than the round, plump shape seen in most people. These sickled cells are sticky and can accumulate in blood vessels, causing severe pain and fatigue, sometimes to the point of hospitalization.

Ten years ago, Nicole also was diagnosed with PH secondary to SCD, which had later manifested as acute chest syndrome, a common, severe complication of SCD. After the PH diagnosis and being told that this chronic illness was progressive and could possibly be life threatening within only a few short years, Nicole defiantly thought to herself, “Yeah right, I’ve heard that before.” She is currently considered blood transfusion dependent because her body no longer reproduces red blood cells properly; this in turn causes her to collect excessive iron (acquired hemochromatosis).

With so many health challenges already, Nicole says PH was “just the icing on the cake” for her, but she has adopted a positive attitude and focuses on the unique opportunities her rare diseases provide her. “Having these diseases has led me to a platform to help educate and discuss SCD and PH, two rare diseases, and to show people, no matter how sick we are, we still have a purpose. We can still live a meaningful and rewarding life.”

After 25 years working first as a legal secretary, then in property management, she decided she would still continue to live her best life; though she no longer works, she spends considerable time and energy contributing to PHA as a PHA support group leader for the Lexington, Ky., group and as a founding member of PHA’s Support Group Leader Advisory Board. Nicole trains and mentors new volunteers and participates in discussions that guide PHA’s support group program. Recently, Nicole also was appointed to the PHA Registry Committee as a patient representative.

Nicole’s motto is, “I will not give up, and I will not give in.” She enjoys traveling, walking on the beach or in the park, playing golf, reading and visiting with the elderly; and she does it all with her faithful toy Yorkie in tow. “My dog was a gift from my late mother after I was diagnosed with PH. He has truly been a godsend, and with that, I have taught him the importance of giving back; he takes pleasure in being a comfort not only to me, but to others as well.”

For more than four decades, Nicole has experienced the physical and emotional challenges of rare diseases, and she has developed a positive, hopeful philosophy that she shares with other people diagnosed with PH, SCD or any other disease. “Living with one chronic, life altering illness can be a challenge, but two? Even so, I’ve never asked, ‘Why me?’ and I do not complain. I feel very blessed to live in a day and age where science and medicine are advancing on a daily basis. We never know what the future holds, and that leaves a lot of room for hope.”

More information about SCD and its relationship to PH is available on the PHA website.

Nicole Creech with her Yorkie, Yeager.