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Riley Hospital treats sickle cell patients' minds with book club

The hospital has given out over 100 books to children living with sickle cell disease as part of the Sickle Cell Story Book Club.

INDIANAPOLIS — A new book club at Riley Hospital for Children at IU Health is bringing together families dealing with a rare disease.

The hospital has given out over 100 books to children living with sickle cell disease as part of the Sickle Cell Story Book Club.

While the disease has been known for over 100 years, doctors specialized in treating sickle cell say it is underfunded because it is rare and affects the minority population. Screening for the disease in newborns is mandatory in every U.S. state, plus the District of Columbia, according to the American Academy of Family Physicians Foundation.

“The results typically come back in a couple weeks,” said Dr. Seethal Jacob, director of the Sickle Cell Program at Riley. 

Jacob is a pediatric hematologist who specializes specifically in sickle cell disease. 

“In sickle cell, there is a mutation that causes the hemoglobin to change shape once the red blood cell has let go of the oxygen,” she said. “That’s where we get the name - it looks like a sickle or half-moon, or a banana."

That mutation is more common in populations hailing from geographical locations where malaria was once prevalent. 

“Africa, Indian subcontinent, parts of South America,” Jacob said, "because it was actually protective."

She said that the body was trying to protect against “the malaria parasite from getting into the red blood cells.” 

Because it is a hereditary disease passed through recessive genes by both parents, it is found in populations who can trace their roots back to those geographical locations. Every racial/ethnic population has people living with sickle cell disease, according to the Centers for Disease Control and Prevention

But “through global expansion, that (malaria-related origin of the mutation) has led to sickle cell disease being more prevalent among Black, Indian and Hispanic populations through the world, and that holds true here in the U.S. as well,” said Jacob. 

But that mutation that occurred to protect populations can cause excruciating pain. 

“The very nature of sickle cell disease affecting hemoglobin that’s inside the red blood cells means it really touches every part of your body,” said Jacob. It’s also one of the reasons how it manifests in each patient is individualized, along with the fact that there are different types of sickle cell disease, the most common being Hemoglobin SS disease. 

Babies are born with sickle cell disease

Two weeks after having her baby, Secili Echols was told her daughter had sickle cell disease. But it wasn’t until Jamiya Ivy’s toddler years “when the sickle started to really kick in,” said Echols. 

“Her cries would be more of a screech than a regular toddler crying, so you could really her the pain,” said Echols. 

“That half-moon or banana shape doesn’t move through our red blood cells as easily as well as the nice round red blood cells do, and it gets stuck in our blood vessels,” said Jacob. 

When those cells get stuck, it causes what patients call a crisis. 

Crisis of pain 

“My teeth start to hurt,” said Ivy. 

That’s how the 13-year-old knows a crisis is looming. But where the pain spreads to is unpredictable. 

“Most often my arms and legs hurt, but sometimes it can be my chest, my head, pretty much anywhere,” said Ivy. 

For 17-year-old Isaiah Farrow, the pain is mostly in his arm. 

“You can have a crisis at any time,” said Farrow. 

He said it’s more common for him to have them in extreme temperatures. Jacob said that’s because certain conditions, including regulating body temperature, require oxygen and can put stress on the body. She said that when working out or playing sports, it’s important for patients to hydrate and “know their limits.” 

“I can tire out easily,” said Farrow, "and it can cause pain crises, and sometimes causes shortness of breath as well."

Farrow said he goes to the doctor monthly. 

“It’s like a catchup on last time I was here. Any improvements, making sure I’m still healthy and that I’m doing what I need to do to improve my health,” said Farrow. “The biggest factor (for maintaining and improving his health) is taking my medicine, Hydroxyurea, Vitamin D, those two medicines to make sure I’m on the right track."

Farrow said he’s been hospitalized for his pain about four times. 

Echols said 2021 has been a difficult year for her daughter. Ivy said she has had a crisis every month. 

“She’ll fight it at home with medicine, her heating pad, she’ll try basically everything - hot bath, shower, everything. And then, once she says ‘Can you please take me to the hospital?’ That’s when I know, you know, it’s bad now,” said Echols. 

Echols said hospital staff will usually draw blood to see where Ivy's numbers are, give her pain medication and run X-rays if her chest hurts.

“But after pain medicine, it’s really just trying to rest and see if she gets any better or when the blood work gets back, they’ll know, ‘Call Riley, this is what we have,’ and they may say transport her,” said Echols. 

That’s how Echols found her daughter's sickle cell team at Riley. Ivy was transported when she was 6 years old. It’s because of this team that Echols, a mother of three, drives three hours for her second-eldest daughter's appointments. 

Riley's approach to sickle cell care

Riley Hospital for Children tries its best to “collocate the resources that our patients would need in one place," Jacob said. "Because when you live with a chronic disease, you often have more than one doctor or more than one team that you need to meet with."

Having to see each doctor in a separate appointment can be disruptive to work, school and extracurriculars. 

When sickle cell patients come for their checkups at Riley Hospital for Children, “they will see a hematologist, they sometimes will see a pulmonologist who comes to clinic as well, they’ll sometimes see a neurologist who does the transcranial dopplers, so those are our stroke screens. They also meet with our psychologist Dr. (Julia) LaMotte if necessary, a social worker, nurses, nurse coordinators, school teachers,” said Jacob.  

Jacob said sickle cell can cause a wide range of “complications in the lungs, in the kidneys, there’s risk of a stroke, even silent strokes that can cause complications at school and make learning difficult. So it really is important to have a multidisciplinary team that your patients and family can really easily access."

“That’s really the model for how sickle cell care should be,” she added. 

Missing school and work

“Sometimes it lasts for days, other times it lasts for hours,” said Ivy. 

“As a parent of a child who goes through something you can’t control, you have to build some type of inner strength just to get through,” said Echols. 

She said she’s relied on her will and faith. 

“Her third grade year, she missed 89 days,” said Echols. “It was a rough year, we will never forget that...89 days, I will remember that because it was like every day, she was sick.

“There were times where I had to pick her up from school and I would have to step into another office or something and like, let the tears flow,” said Echols. “Coming to the school, thinking she would be OK throughout the day, and I have to turn around and pick her up from the school."

She said work is rough, as well, having to take time off to care for her daughter.

This year, Echols was offered a new position, but 2021 also marked a difficult year for her daughter's health, with major crises in January and May. 

“I was offered a new position, but she was getting sick so often that, that position I had to turn down because there was no guarantee, you know, I didn’t know, I couldn’t say ‘she’s not going to be sick,’” said Echols. “At that time, she was getting sick back to back to back, and I had to say ‘At this time, I cannot take this position, even though I want it, and I feel like it will financially help my family, it’s just...at the time she’s getting sick so often, I don’t want to accept (the job),' and boom, we’re in the hospital, and I come back, and I don’t have a job."

Echols said she has been with her current employer “for a while,” so “they’re more understanding when I say ‘I have to go, I have to go, and I don’t know when I’ll be back.' And they do the best they can with staff, and I just update them every day with what’s going on in the hospital." 

“It’s rough on finances,” said Echols. 

Ivy and her mom live in Hammond, Indiana. Riley Hospital for Children is where Ivy’s care team is, but when she’s in a crisis, they head straight to their local hospital. 

“Since we live so far away, I usually call here (Riley Hospital For Children) before we go to our local ER so that we don’t run into any problem, what to draw, what to look for, to basically give them the rundown of what they should do” said Echols. 

As many parents will tell you, Echols has had to become her daughter's advocate when it comes to Ivy’s health. 

“I had to teach myself everything,” said Echols. “It is frustrating sometimes because you have to repeat yourself, or you get doctors or nurses, usually nurses, who just came out of nursing school and the question they’ll say is ‘When did she get sickle cell?’” 

“It frustrates you,” said Echols, because babies are born with sickle cell, they don't "get" the disease. 

Amid the excruciating pain that Ivy said she feels monthly for this 13-year-old, the pain is difficult - but isn’t the hardest part. 

“The hardest part is telling people that I have sickle cell because a lot of people don’t know what it is,” said Ivy. 

The ignorance of others can make their reactions unpredictable. But people unaffected directly by sickle cell disease aren’t the only ones who need to know more about it.  

“There are a lot of aspects of how different types of sickle cell disease appear within each individual that we still don’t know, which goes back to the need of research and understanding about that,” said Jacob. 

Where's the money for research?

Sickle cell disease was identified over a hundred years ago “and to only have four FDA-approved medications, three of which happened after 2017, is pretty devastating for the population that we have here in the U.S.,” said Jacob. 

“We’ve been really behind in the research and development of drugs for sickle cell disease,” she added.

Jacob said the reason for the lack of development and research comes down to “who sickle cell affects in the U.S.” 

“Sickle cell is a rare disease, but it’s a rare disease that affects minority populations,” said Jacob.

“The initial funding for sickle cell centers in the 1970s that created several comprehensive sickle cell centers around the country was eventually let go, and many of those centers closed,” said Jacob. “And so we have a population of patients, where the majority don’t get care at a comprehensive sickle cell center, and for a chronic disease that has many complexities to it, that really shouldn’t be how we approach their health and their long-term care."

Sickle Cell Story Club 

Part of Riley Hospital For Children’s take on comprehensive care is coming to life through a new initiative in the Sickle Cell Center. 

“We have the privilege of taking care of them, and it’s not just about their physical health — it’s about their emotional and academic health as well,” said pediatric psychologist Dr. Julia LaMotte. 

LaMotte is an assistant professor of clinical pediatrics, and she recently launched the “Sickle Sell Story Club” because “we really wanted to promote reading.” 

“Every time a family comes to an appointment, they get a brand new book. And we have selected books that have characters that represent the families that we serve,” said LaMotte. “So, they predominately have Black protagonists with storylines that promote things like self-esteem and feeling good about who you are in the skin that you are in.". 

“Representation matters, and this is particularly true for children who struggle with reading,” said LaMotte.  

Neither Farrow or Ivy appeared to struggle with reading. In fact, both are avid readers. Amid their missed days at school due to their health, both Ivy and Farrow have been able to keep up with school at their appropriate grade level. 

“Oh, I love reading,” said Ivy as she was presented two books to choose from. Ivy said she loves history, writing and reading. 

“I’m a big reader myself,” said Farrow. The rising high school senior, who aspires to be a journalist, said he has a library at home and loves reading books by Richard Engle. 

“It’s a good thing to have a good variety of different books,” said Farrow. 

“I think it’s really great to see their joy, knowing that we’ve thought of them, when we hand them the book. I think it personalizes the program,” said LaMotte. 

LaMotte said the hospital takes monetary and book donations for the club.

“We have selectively curated a list of books that we think are appropriate for this program in particular, but we are currently working to establish a fund specific to the Sickle Story Fund," she said.

If you are interested in supporting the Sickle Cell Story Club, you can submit your donation via this link, and just make sure to specify where you would like your donation to go. 

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