Sickle cell disease takes a hidden toll on fertility : Shots


Teonna Woolford, 31, in Owings Mills, Maryland on November 4, 2022. Woolford has sickle cell illness and has been unable to afford fertility preservation for years.

Rosem Morton for NPR


disguise caption

toggle caption

Rosem Morton for NPR


Teonna Woolford, 31, in Owings Mills, Maryland on November 4, 2022. Woolford has sickle cell illness and has been unable to afford fertility preservation for years.

Rosem Morton for NPR

Teonna Woolford has all the time mentioned that when it got here time to construct a household, her very best could be to have six youngsters.

Why six?

“I do not know the place that quantity got here from. I simply felt like 4 wasn’t sufficient,” says Woolford, a Baltimore resident. “Six is an effective quantity.”

Woolford, 31, was born with sickle cell illness. The genetic dysfunction impacts sufferers’ blood cells. They turn into misshapen, which makes it tougher for the blood to hold oxygen and circulation all through the physique. This will trigger strokes, organ harm and frequent bouts of excruciating ache.

Sickle cell illness impacts an estimated 100,000 individuals within the U.S. and the overwhelming majority of them are Black. Federal and charitable {dollars} devoted to sickle cell pale compared to different much less frequent illnesses that have an effect on a majority white sufferers.

KHN logo

Physicians and researchers say the illness is a stark instance of the well being inequities which pervade the U.S. well being system. A poignant expression of this, affected person advocates say, is the silence across the impression on fertility, and lack of reproductive and sexual well being look after the younger individuals residing with this advanced illness.

Crushing hopes for fertility

Woolford has lived by way of your entire gamut of sickle cell issues. By 15, her hip joints had turn into so broken that she needed to have each hips changed. She was depending on frequent blood transfusions to scale back ache episodes and vascular harm, and her liver was failing.

“So many issues, infections, hospitalizations, and so by the point I graduated highschool, I simply felt defeated [and] depressed,” says Woolford, who first spoke with an NPR reporter from a hospital mattress in Baltimore, Maryland. She’d skilled a sickle cell ache disaster a number of days earlier, and was nonetheless receiving ache remedy and intravenous fluids.

In her late teenagers Woolford sought out a bone marrow transplant: a remedy that allows sickled cells within the affected person’s physique to get replaced with wholesome cells from a stem cell donor. The process comes with dangers, and never everyone seems to be eligible. It additionally depends on discovering a appropriate donor match. But when it really works, it could actually free an individual from sickle cell illness ceaselessly.

Woolford could not discover a excellent match, so she enrolled in a medical trial the place medical doctors may use even a half-matched donor.

As a part of the bone marrow transplant, sufferers are first uncovered to chemotherapy, which might impair or get rid of fertility.

Woolford hesitated. In any case, her very best household included six kids.

Woolford has sickle cell illness and has been unable to afford fertility preservation for years. She began a non-profit to supply monetary grants for sufferers who want fertility care, and advocates for extra consciousness concerning the results of sickle cell on sufferers’ household planning and sexual well being.

Rosem Morton for NPR


disguise caption

toggle caption

Rosem Morton for NPR


Woolford has sickle cell illness and has been unable to afford fertility preservation for years. She began a non-profit to supply monetary grants for sufferers who want fertility care, and advocates for extra consciousness concerning the results of sickle cell on sufferers’ household planning and sexual well being.

Rosem Morton for NPR

When she advised her physician about her fear, his response crushed her. “This physician, he checked out me and he was like, ‘Properly, I will be sincere, with all of the issues you’ve got already had from sickle cell, I do not know why you are even nervous about this course of making you infertile since you’re most likely already infertile.'”

Even with no transplant, sickle cell illness can harm sufferers’ our bodies in methods that may have an effect on their capability to have kids, in response to Dr. Leena Nahata, a pediatric endocrinologist at Nationwide Kids’s Hospital in Ohio.

For girls, persistent irritation and sickling of blood cells within the ovaries could make it tougher to get pregnant. For males, sickled blood can jam contained in the blood vessels of the penis, and that may trigger painful, undesirable erections which final for hours. This situation, known as priapism, can harm sexual operate and reduce sperm depend.

And it isn’t simply the illness. Researchers are evaluating how some widely-used remedies might have an effect on fertility — for instance by lowering sperm depend.

“It stays unclear how that interprets on to fertility outcomes, however a minimum of raises the priority that this can be a problem,” Nahata says.

Much more regarding to Nahata had been the outcomes of a small examine she co-authored that confirmed some sufferers are unaware of the numerous fertility dangers associated to sickle cell illness.

Would hope for a remedy imply giving up on fertility?

Woolford was solely 19 when her physician shocked her by telling her she was most likely already infertile. However nobody may make certain, so she held out hope that she would possibly nonetheless bear a process to protect her fertility, earlier than present process the chemotherapy required for the bone marrow transplant.

After intensive analysis, she realized egg freezing may price greater than $10,000, her insurance coverage would not cowl it, and she or he could not afford that on her personal.

Woolford puzzled if perhaps she may discover one other technique to pay for egg freezing. “So I began trying into monetary assets. And I noticed all these foundations [that] give away grants. However you needed to have a analysis of most cancers,” she says.

“So lengthy story quick, I went by way of with a [bone marrow] transplant, sort of feeling like if I used to be going to be cured from sickle cell, it would be a good tradeoff to surrender my dream of organic kids.”

Sadly, the partial-match transplant didn’t work. Woolford’s physique rejected it.

“So, right here I’m. I’m 30, nonetheless have sickle cell illness and I am infertile,” she says.

A grim thought nonetheless generally pops up in Woolford’s thoughts: Possibly if she had most cancers as a substitute of sickle cell, her goals of getting organic kids may nonetheless have come true.

How systemic racism impacts entry to care

The primary description of sickle cell illness in medical literature was revealed over a century in the past. As a result of the overwhelming majority of sickle cell sufferers within the U.S. had been Black, it rapidly grew to become labeled as a “Black illness,” and with that got here a legacy of systemic racism that also impacts sufferers at this time.

Black sufferers are inclined to have much less social capital and fewer assets, in response to Dr. Lydia Pecker, a sickle cell illness researcher and assistant professor of medication at Johns Hopkins College. Pecker has studied fertility in sickle cell illness, and says there is a stark distinction between assets out there to most cancers sufferers versus sickle cell sufferers, relating to fertility remedy.

“There are any variety of foundations, massive and small, that assist assist and pay for fertility preservation for individuals with most cancers,” Pecker says. “These foundations really work with fertility preservation facilities to barter decrease charges for affected individuals.”

There are clear medical tips stating kids with most cancers going by way of chemotherapy needs to be referred for fertility preservation.

Kids with sickle cell illness going by way of transplants are uncovered to chemotherapy too, “however we do not actually have tips like that but for individuals with sickle cell illness,” Pecker says.

It is not an ideal comparability, she provides, as a result of the sorts of chemotherapy medication utilized in pediatric most cancers are completely different from the chemotherapies utilized in sickle cell remedy. However fertility preservation could be essential when there may be any danger of treatment-associated fertility impairment. With out clear and extensively adopted medical tips, sickle cell sufferers might not get referred to acceptable care.

Pecker says present medical apply forces sickle-cell sufferers to make a troublesome selection: “Like, you may have remedy or you may have fertility. However what we are saying in most cancers care is you may have remedy and you may have fertility.”

Costly remedy and combating for entry

“Traditionally, we within the sickle cell neighborhood have actually needed to give attention to survival over high quality of life. Now, because of advances in science and the resilience of the neighborhood, we live longer. Now, it isn’t sufficient to outlive. For lots of us, having an optimum high quality of life includes having kids.”

Rosem Morton for NPR


disguise caption

toggle caption

Rosem Morton for NPR


“Traditionally, we within the sickle cell neighborhood have actually needed to give attention to survival over high quality of life. Now, because of advances in science and the resilience of the neighborhood, we live longer. Now, it isn’t sufficient to outlive. For lots of us, having an optimum high quality of life includes having kids.”

Rosem Morton for NPR

Within the U.S., medical insurance protection for fertility preservation and remedy will not be assured and varies from state to state. Solely eleven states have legal guidelines that mandate protection for fertility preservation for sufferers who bear remedies that might imperil their capability to have organic kids – normally known as iatrogenic remedies – like chemotherapy or radiation.

However even these legal guidelines do not all the time assist, says Dr. Irene Su, an OB-GYN on the College of California San Francisco, who researches fertility preservation insurance policies.

Su says these legal guidelines are normally very imprecise when it comes to who qualifies: “When you give insurers room to interpret, it is doable that they are often very broad, or they are often very slim.”

Which means that many sickle cell illness sufferers who haven’t got this protection are compelled to pay out of pocket.

Phillip Sanders of Arkansas was a sophomore in school when his sickle cell illness issues ramped up.

“By 2016, that entire 12 months, I spent 60 p.c of my life within the hospital,” he says.

Not lengthy after, he married his school sweetheart. They wished to have kids, however had been unable to get pregnant. A health care provider then advised them their greatest guess was to bear intrauterine insemination, a fertility remedy the place the highest-quality sperm is chosen and inserted immediately into the uterus.

Sanders’ spouse obtained pregnant after 4 makes an attempt, however none had been coated by insurance coverage.

“It is so costly. You are speaking about 1000’s of {dollars} every attempt,” Sanders says. “I imply, we needed to, in fact, borrow cash. As a result of, you already know, on the similar time, I used to be going by way of faculty, opening a enterprise, and I used to be within the hospital, all the time. So it was extraordinarily powerful.”

Like Woolford, Sanders enrolled in a medical trial for a bone marrow transplant, and it was successful.

He’s now 31, is sickle cell illness free and has a son, Phillip, Jr.

Teonna Woolford for her half, has needed to give attention to different objectives. After her transplant failed, the continuing toll of the illness on her physique continued. She has needed to come to phrases with the impossibility of her ever having a organic youngster of her personal.

Woolford launched a brand new nonprofit, The Sickle Cell Reproductive Well being Training Directive, to lift consciousness of fertility points at medical conferences and amongst sufferers. A future aim for the group is to supply monetary grants to sickle cell sufferers struggling to pay for fertility preservation and coverings.

Most days, Woolford finds the work empowering. On different days, she admits it reminds her of the awful private actuality that she is going to most likely by no means conceive a toddler.

“It is actually arduous, as a result of I do not suppose lots of people understand that I am combating for one thing that I did not have entry to,” she says.

At this level, she mentioned, it is not a medical justice combat. It is a reproductive justice one.

Reporting for this story was supported by the USC Annenberg Heart for Well being Journalism’s Impression Fund for Reporting on Well being Fairness and Well being Techniques. It was produced in partnership with Facet Results Public Media, WFYI and KHN (Kaiser Well being Information).