Sickle cell anemia

Toddler Kaliyah and her family find treatment, comfort at UW Health

Toddler Kaliyah Nelson being held in a parents' arms

Your heart really has no choice. It simply melts once you meet a precious little girl named Kaliyah Nelson of Janesville, Wis.

As a descriptor, “adorable” doesn’t really do this 2-year-old child justice. Her infectious smile, big eyes and sweet personality are impossible to resist.

Kaliyah gives everyone she meets immense pleasure. Sadly, however, this delightful toddler has experienced all too much pain. Born in July 2019 and diagnosed on Day 4 of life with sickle cell anemia, Kaliyah was symptom-free until she was 5 months old.

“That’s when her energy level dropped, she stopped eating and everything flared up,” said Kaliyah’s mother, Shyniece Jackson.

An inherited red blood disorder, sickle cell anemia is named after the abnormally shaped red blood cells whose crescent shape resembles a sickle harvesting tool. The genetic sickle cell trait – not the disease itself – is found in about 10 percent of all African Americans in the United States, although it is also found in Hispanics, South Asians, Caucasians from southern Europe, and people from Middle Eastern countries. If both parents have the trait, each child has a one in four chance of getting sickle cell anemia.

“Some children, like Kaliyah, have more severe symptoms and some less so,” said UW Health Kids pediatric hematologist/oncologist Carol Diamond, MD, who cares for Kaliyah.

“Normal blood cells are disc shaped, so they flow freely throughout the bloodstream for most of us. People with sickle cell anemia get sick because the abnormally shaped sickle cells tend to get stuck inside blood vessels,” she said. “When this happens, oxygen delivery to tissues is compromised.”

Many symptoms of sickle cell anemia

Depending on the severity of sickle cell disease, said Dr. Diamond, symptoms can include:

  • Fatigue – a result of the anemia, or shortage of red blood cells

  • Pain crises that can last hours or weeks; these frequently occur in the chest, abdomen, joints and bones

  • Swelling in the hands and feet, also caused by insufficient blood flow

  • Frequent infections due to a patient’s spleen not working well or at all

Sickle cell anemia is only cured by bone marrow transplant – often an unavailable option – but patients can grow and thrive normally with proper medical care and a supportive home environment. Fortunately, new gene editing therapies – such as one called CRISPR – are showing great promise.

“Stress management is incredibly important for these kids,” said Dr. Diamond. “This is a tough disease for any child and family, but it’s a lot more manageable when there is a safe, stable home environment.”

Over the past year, Shyniece has taken this to heart and tried to make her daughter’s life as normal as possible, notwithstanding several hospitalizations and blood transfusions.

“It’s not easy as a single parent,” she said, noting that her other daughter, LaZaria, 9, also needs her share of motherly attention. “It can be heartbreaking at times, but normally Kaliyah is such a happy, active and smiling child.”

From mid-2019 through mid-2020, Kaliyah experienced a steady stream of pain crises. As hard as she tried, Shyniece’s ability to handle the stress of the situation began to wane.

While not a cure, spleen removal could help

By Fall 2020, Dr. Diamond and her team at UW Health’s American Family Children’s Hospital sat down with Shyniece to discuss the idea of removing Kaliyah’s spleen, an important organ located in the upper left part of the belly.

“The spleen helps fight infection by filtering germs out of the bloodstream,” said Dr. Diamond. “With children like Kaliyah, the sickle-shaped cells get trapped in the spleen, causing that organ to enlarge and, in turn, trigger anemia and pain. Over time, the spleen eventually shrinks and dies. For Kaliyah, we recommended removal of her spleen as a way to prevent dangerous episodes of worsened anemia and keep her out of the hospital."

After talking with Shyniece, Dr. Diamond referred Kaliyah to UW Health Kids pediatric surgeon Charles Leys, MD, who performed the surgery – called a splenectomy – at American Family Children’s Hospital in early November.

“Kaliyah did great with the surgery,” said Dr. Leys. “We do this procedure laparoscopically through a small incision in her belly button so she won’t have a visible scar. She was doing well and her belly button looked completely normal when I saw her a couple of weeks after surgery.”

Penicillin will help fight off infections

The spleen stops working normally in all children with sickle cell anemia early on, so they start taking penicillin upon diagnosis. To compensate for her missing spleen – one of the body’s natural infection-fighting organs – Kaliyah will stay on daily penicillin for the rest of her life. She also received several vaccines before the surgery to help boost her immunity.

“They told me kids who have their spleen taken out are typically older than Kaliyah, but it got to the point where I just couldn’t see my child in such pain anymore,” said Shyniece. “Taking out her spleen is not a cure, but she is a lot more like her regular self.”

Thinking back to when Kaliyah was born, Shyniece thought about moving back to her hometown of Chicago to be closer to family. Once her baby was diagnosed with sickle cell anemia, however, Shyniece decided that being close to her UW Health Kids care team was in her daughter’s best interest.

“They know Kaliyah from A to Z in Madison,” Shyniece said. “When I call and tell them she isn’t smiling, they know something is wrong and help me figure out what to do next.”

Kari Weier, NP, a UW Health Kids pediatric hematology/oncology nurse practitioner who works with Dr. Diamond, cherishes how valuable these long-term relationships are to each family.

“We see these patients at their worst and at their best, so this allows us to provide great reassurance when a mom like Shyniece is on the phone not sure what to do,” Weier said.

For Shyniece, getting through each day is a major juggling act, so a comforting voice like Kari’s can mean a world of difference.

“It’s the greatest, most comforting feeling,” Shyniece said. “Because they know Kaliyah and me so well, I can rest easier after checking in.”