Karen's Story: A Second Chance for a New Beginning
There is no specific reason why Karen got the inflammatory disease, sarcoidosis. It’s idiopathic, meaning doctors haven’t been able to pinpoint a definitive cause. For the majority of people with sarcoidosis, they don’t suffer any symptoms and the disease clears up by itself. For Karen, it was different: It led to the complete deterioration of her lungs and heart. This is her story.
A Diagnosis of Sarcoidosis
When Karen was 40 years old, she noticed the skin in the area of her appendix was no longer supple and seemed to be hardening. She’d had her appendix removed nearly 30 years prior, and there didn’t seem to be anything else that was wrong. Rightfully concerned, she went to her dermatologist, who took a biopsy.
The dermatologist found that Karen had sarcoidosis, a disease in which inflammation occurs in certain organs of the body, most commonly in the lungs or lymph nodes. Karen saw the inflammation through the hardening of her skin.
Doctors think that sarcoidosis may be an immune reaction to an infection that then persists even after the infection is cleared from the body. In most cases it goes away on its own, but in some cases sarcoidosis can get worse and become life-threatening.
Karen’s doctors decided to wait and see if it went away, but years later she experienced mucus in her chest and had trouble breathing. She went to Penn Medicine pulmonologist, Morris Swartz, MD, at Penn Presbyterian Medical Center. Dr. Swartz took a chest x-ray and found that the sarcoidosis had spread from her skin, and she now had scar tissue growing in her lungs. Because of its effect on her lungs, she needed to start wearing oxygen.
A Diagnosis of Pulmonary Hypertension
Three years after she went on oxygen, Karen felt very sick and went to the emergency room. Doctors discovered that the scarring and lack of air getting to her lungs had resulted in pulmonary hypertension. Pulmonary hypertension is abnormally high blood pressure in the arteries of the lungs. It starts when tiny arteries in your lungs become narrowed or blocked, making it harder for blood to flow through your lungs. As the pressure builds, the right side of the heart has to work a lot harder than normal to pump blood through the lungs. The pressure caused Karen’s heart muscle to weaken and fail.
“I was told by the specialist that one more day and I probably wouldn’t have woken up the next day,” says Karen.
After being hospitalized for two weeks, Karen left with a catheter in her chest, a canister that she had to wear to administer medication, and an oxygen cart.
Her quality of life significantly declined.
“I was on 10 liters of oxygen and, for anyone who doesn’t know, to be on two liters is a high level,” explains Karen. “To be on 10 meant that, basically, I couldn’t go out of the house 'cause the pushcart canister ones only go up to six. … It was arduous even to go to the bathroom with all of this equipment hooked up to me. … If I went out, I couldn’t do anything; I couldn’t walk anywhere. I had to get back home and mix the medicine 'cause you have to have that on 24 hours a day. If you run out or if the canister breaks, you have to hurry up and change it. I had to retire from my job as a real estate agent.”
Her lung transplant pulmonologist, Vivek Ahya, MD, told Karen that her five-year prognosis didn’t look good and encouraged her to get a transplant, but she refused.
It was when she went to Good Shepard Penn Partners for rehabilitation that she saw the light.
“One of the things they encourage you to do [after you have a transplant] is to stay healthy and to exercise. When I went to rehab, I saw people [who had just had a transplant] come in. They were maintaining themselves well after the transplant, and I thought, ‘I'm getting more sick, and the [rehab’s] not helping. There's not going to be a cure for pulmonary hypertension yet.’”
She decided to go on the waiting list.
“Mommy’s awake! Mommy’s awake!” were the first words Karen heard.
She had been on the waiting list for a lung transplant for two years before she received the call to come in to Penn and have surgery.
The transplant had complications. Karen experienced internal bleeding; her body was swelling and holding water. Doctors placed her on a machine called extra corporeal membrane oxygenation (ECMO) that oxygenates the lungs, giving them a chance to recuperate while they’re trying to get rid of fluid and swelling. On ECMO, with doctors working to save her life, Karen slept in an induced coma for 16 days.
When she finally woke up, her family surrounded her.
Her lung transplant surgeon, Edward Cantu III, MD, told her, “Karen, I did everything I could. You were not going anywhere. I was going to make you all okay.”
And she was okay.
A New Beginning
After her transplant, Karen had a long road to recovery with a lot of therapy. Atrophy had set in from the coma. She also had to relearn how to do daily tasks – things most people take for granted like how to make a bed, turn on a stove to cook, walk up the stairs.
Today, she is careful not to expose herself to certain agents so she doesn’t get the flu or a cold. She worries about lung rejection. She works hard to keep herself healthy. But it’s worth it.
“I can do just about everything I could before I got sarcoidosis,” she says. “I can go up steps, walk, hold my grandbaby. I just became a grandmother four months ago – my first grandchild.”
Karen’s advice to others considering going on the waiting list:
“Be proactive. Keep yourself in good shape and eat correctly. If you’re afraid, speak to someone. Penn's Harron Lung Center and Lung Transplant Program have a wonderful support system from the doctors to the nurses to the support groups to the patients. Speak to a specialist there. And take the chance because you won't regret it.”
An empty oxygen tank sits in Karen’s house as a reminder of the past and as a motivator to keep herself healthy.
If you're suffering from lung disease, make an appointment with Penn's lung program.