Story by Lori Newman on 03/18/2019Imagine being alone at home lying in bed, your loved one hundreds of miles away and you're experiencing debilitating pain in both legs. You call 911 for help, and crawl down a flight of stairs so paramedics don't have to break down the door.
This is exactly what happened to 36-year-old Alexis Piper, who has been battling sickle cell anemia since she was 7 years old.
"The pain in both my legs was severe," Piper said. "It kept getting worse. I couldn't move my legs. I couldn't walk."
Sickle cell anemia is an inherited form of anemia, in which there aren't enough healthy red blood cells to carry adequate oxygen throughout the body. With sickle cell anemia, the red blood cells become rigid and sticky and are shaped like sickles or crescent moons. These irregularly shaped cells can get stuck in small blood vessels, which can slow or block blood flow and oxygen to parts of the body.
People who have sickle cell anemia often experience pain and other serious problems, such as infection, lung-related complications and stroke.
"I have noticed that as I get older the episodes have decreased, but they have become more severe when they do happen," Piper said. "The pain hits in random places of the body. It may start as a slight throbbing and then intensify to a sharp pain."
Piper's husband, Air Force Master Sgt. Antwan Piper, had left that morning for a business meeting out of state. He was having dinner with his coworkers when he received his wife's phone call.
"Talking to her on the phone was so hard," Antwan said, remembering his feeling of helplessness. "It hurt me even more, because she crawled from upstairs to open the door. I could picture her crawling just to open the door. Words can't even explain that."
Once Alexis arrived at Brooke Army Medical Center the doctor's began treating her with the normal protocols for a patient with the disease.
"I thought it would be a simple case of her just needing intravenous fluids, pain medications, and potentially a blood transfusion," said Air Force Capt. (Dr.) Lauren Lee, the hematology/oncology fellow who was on the inpatient service the next morning. "It's not uncommon for sickle cell patients to come in with a lot of bone pain. We treat them supportively, sometimes with a blood transfusion, and they go on their way. That's what I expected with Alexis."
Alexis was given a blood transfusion, but her body did not react as expected.
"We noticed that her blood counts didn't change," Lee said. "In the absence of bleeding, normally we would expect to see the hemoglobin rise after a unit, but hers didn't improve."
She had developed a rare condition called hyperhemolysis.
"With hyperhemolysis, the body not only breaks down all of the transfused blood, but it begins to breakdown the patient's own blood and this is what happened to Alexis," Lee explained.
"Approach to therapy is challenging because it's such a rare condition," Lee added. "Generally speaking these patients respond to steroids and intravenous immunoglobulins (antibodies)."
It became clear after about three days that treatment was not working. At this point Alexis' hemoglobin count was about three. A person with sickle cell anemia normally has a hemoglobin count of seven to eight. A healthy person's normal hemoglobin range is 12 to 14.
After days of steroids and IV immune globulins, the doctors decided to give her an additional unit of blood, but about 20 minutes into the transfusion Alexis started experiencing severe pain and her hemoglobin continued to decrease. She started to become confused and showed evidence of heart failure.
"Things were only getting worse, by the minute really," Lee said. "A hemoglobin of two is essentially incompatible with life."
"Somehow Alexis survived, but her body could not tolerate this for a prolonged period of time," she added. "Her heart was working very hard and she was not getting enough oxygen to her brain."
During this time, Antwan and Alexis' family were at the hospital night and day hoping and praying for a miracle.
"Every day we were hoping, but every day things were decreasing, not getting better," Antwan said. "During that time, I really, really depended upon my faith. No one had any answers about what she was going through."
Lee began researching other treatment options. She contacted an expert in England, Dr. Win Nay, who had published extensive research about sickle cell patients with hyperhemolysis.
After discussing the treatment options with a multidisciplinary team of doctors and Alexis' family, they decided to try an off-label drug primarily used to treat other conditions as such rheumatoid arthritis.
"This was a situation where we clearly had to think outside the box," said Air Force Maj. (Dr.) Brad Beeler, hematology/oncology fellow, Alexis' primary hematologist.
The drug was successful in stopping Alexis' body from breaking down the red blood cells; however, her body was not producing new red blood cells fast enough to recover.
This is when the use of bovine-hemoglobin came to mind, because of the extensive research being done on the use of hemoglobin-based oxygen carriers for injuries received on the battlefield.
Lee reached out to one of her former professors, Dr. Mark Zumberg, a sickle cell specialist at the University of Florida. He described a similar case in which they supported a patient with a hemoglobin-based oxygen carrier. Currently the medication is approved for use in South Africa, but is only available under investigational status through the Food and Drug Administration in the United States.
"Within 24 hours we were able to get FDA compassionate release and hospital approval to use the drug and had it flown in from across the country," Lee said.
Alexis was given four infusions of the drug.
"Her response was pretty remarkable," Lee said. "She went from being non-respondent to lucent."
However, shortly afterwards Alexis started having seizures that weren't responding to the typical medicines.
"In order to control the seizures we had to put Alexis into a medically-induced coma and insert a breathing tube for support," said Air Force Capt. John Hunninghake, pulmonary and critical care fellow. "That was a really difficult conversation to have with her husband [Antwan] over the phone in the middle of the night."
Antwan now had to make medical decisions for his wife, because she was not able.
"That rocked my world, because things were starting to get better," Antwan said. "I was feeling defenseless because I couldn't do anything for her. She was laying there going through this and all I could do was watch and hope and pray. It was tough."
After talking with the doctors, Antwan agreed to keep treating her with the drug, because he believed that if the treatment didn't work for her, the lessons they learned could possibly help someone else.
"Her family was exceptional in this situation," Lee said. "They never once doubted our ability, they had faith and they continued to encourage every one of the providers. They kept a positive outlook the entire time."
Miraculously, after seven infusions of the drug, Alexis made a full recovery.
Antwan praised the doctors for their compassion, "They would answer all of our questions. Take time to pray with us. To give me a hug. To cry with me. What doctor would do that?
"I don't consider them just doctors, I consider them now my friends," he said. "That's beyond a doctor. That's the love they had. That's the love we have for them."
"They were all understanding, not just from a medical perspective, they stood down and got to my bedside with me and helped me journey through this," she said. "I'm very appreciative for all of them. The whole team."
Alexis described hearing Lee's voice each morning and Beeler coming to see her each evening.
"I remember him (Beeler) saying, it's not a setback it's just an obstacle,'" she said tearfully. "I began to call him my doctor angel. When I was having a bad moment or was not feeling well he would come and give a medical opinion or medical advice. It would be just what I needed to hear at that moment."
The Pipers continue to maintain a close relationship with Alexis' doctors.
"I am a miracle," said the self-proclaimed sickle cell warrior, survivor and military spouse. "My health and strength are back. I'm stronger now actually, in more ways than one."