We had the pleasure of speaking with Dr Laura Bonebrake, mother of four children and an obstetrics and gynaecology (ob-gyn) physician affiliated with multiple hospitals in the Fenton, Missouri area. As part of her role, Dr Bonebrake treats women with intrahepatic cholestasis of pregnancy (ICP), a rare liver disorder that occurs in pregnant women when bile stops flowing correctly through the liver and builds up in the bloodstream, causing risk to the unborn baby. As a physician who has experienced ICP herself, Dr Bonebrake has a unique understanding of the disease. Here, she shares her own cholestasis journey with us and explains how this has inspired her to become an advocate for the ICP community. Dr Bonebrake is a board member for ICP care, a non-profit organisation with a mission to provide patient support and education, raise public awareness, support the advancement of research and improve health care practices

Laura’s experiences with intrahepatic cholestasis of pregnancy

During her last year of medical training at the Creighton University School of Medicine, Laura became pregnant with her eldest son, Evan. “I was about 23 weeks pregnant when I had a case of pneumonia and was admitted to hospital. They noticed my liver functions were a little elevated, but they didn’t think much of it, and I went home,” Laura explains. “It was about a week later that I started having the itching. It began between my fingers and then became more generalised, getting worse in the evenings.”

Pruritis is an itch or sensation that makes a person want to scratch—it is one of the noticeable symptoms of ICP; it predominantly affects the hands and feet but can be all over the body.

Knowing she had an elevated liver function and now the itching, Laura put two and two together and contacted her ob-gyn to discuss the possibility of ICP: “We did a total bile acid test to check my levels, which came back as high, and from that made a diagnosis.”

Laura’s relatively straightforward journey to diagnosis is unusual in the ICP community. “As an ob-gyn, you’ll maybe see a case every couple of years. We now have some really good guidelines, which has helped to speed up diagnosis, but I have heard of a lot of women who have waited several weeks to get a diagnosis and therefore lost a lot of time to get proper care in place.”

During her pregnancy, Laura found the physical symptoms of ICP to be difficult to manage, particularly with the itching and insomnia that cholestasis of pregnancy can cause. With the help of her medical team and symptom management treatment, Laura feels blessed that the physical symptoms did not dominate her entire pregnancy.

“The physical part of ICP can be bad, but for me the emotional journey was far worse.”

“My emotional journey has been the greater part, and this is not talked about a lot,” Laura shares. “Although it is a rare risk, there is an increased risk of stillbirth with ICP. It is usually a sudden stillbirth that cannot be predicted or prevented.” Laura found it challenging to enjoy her pregnancy knowing the potential risks and worrying about her unborn child’s safety. 

Laura and her husband went into their following pregnancies knowing the recurrence risk for ICP is 60–90% and that Laura might have to deal with the physical and emotional burdens all over again.

“It was a big, very personal decision, as it is for everyone in the ICP community who is thinking about pregnancy again. A lot of ICP moms say they can’t emotionally do it again, which I completely understand.”

Laura was hopeful that she could possibly be the 10% who don’t have a recurrence. “With my second pregnancy, I tried to change my diet, drinking kale smoothies each day in the hope of preventing it, but it didn’t work. I was diagnosed at around 16 weeks when I was pregnant with Colin.”

Laura’s third pregnancy was the most severe case of ICP she experienced: “I was diagnosed at eight weeks with very high levels. They put Elise on a stillbirth risk of 4–5%; she was probably our most emotional pregnancy.” During this pregnancy, Laura had a maternal foetal medicine (MFM) doctor who helped monitor the pregnancy as it developed. “We did a foetal echocardiography to look at her heart, as some studies show that this may help predict a stillbirth. We did daily monitoring for 11 weeks up until she was born,” Laura explains.

All of Laura’s diagnoses with ICP came very early in pregnancy. With Corinne, her youngest daughter, the diagnosis came at just five weeks. At the point, Laura was already experiencing the tell-tale symptom of itching between the fingers.

Laura says that all four children needed support in neonatal intensive care (NICU), and this was probably because of their exposure to ICP, but “they are now all healthy and amazing”. “I was fortunate to have a good, supportive medical team and a supporting family by my side.”

ICP Care: dedicated to protecting pregnancies, families and their unborn children from ICP

ICP Care was founded by Donna Benavides, who lost her first born, Jorden, in 2003 to cholestasis. This patient-led organisation was founded to raise awareness of ICP, improve healthcare practices, facilitate the advancement of research and provide support to pregnant mothers with ICP and those who have lost their child to this disease.

“ICP Care is a great organisation to be involved with.”

Laura discovered ICP Care in 2014 when she was an ICP patient for the second time during her pregnancy with Colin. “I joined them, and it was so nice to see other moms giving support and sharing little tips. Just last night I saw a post about using a hairbrush to itch, and I went to my husband and said, ‘Look, these people are really creative!’” In 2020, Laura joined the ICP Care medical advisory board and the board.

With fellow board member Hilary Boyer, Dr Bonebrake has updated the ICP website, using the platform to address unmet needs and questions from the ICP community. “Moms would ask, for example, what do I do after pregnancy? So, we created a section on the website with research information to help answer this question.” The organisation has also worked hard to listen to the community, creating helpful, educational resources they can refer to, including healthcare brochures and a short, educational video that summarises everything you need to know about ICP in five minutes. Laura and the ICP Care team hope that this video will reach physicians globally and help raise awareness of the disease to improve healthcare practices. 

Laura talks us through three key areas for her and the ICP Care team as they raise awareness about the condition within the healthcare and patient community to facilitate change: 

1. There is an urgent need to streamline information surrounding bile acid testing and diagnosis

“There are a lot of issues around bile acid testing, particularly in the US,” Dr Bonebrake explains. “Here, we have anywhere between a three- to ten-day turnaround for bile acid testing. This means women are waiting for their results not knowing their levels and the danger their unborn child could be in. This is a problem. Testing needs to be expedited.”

Dr Bonebrake explains that there is a lot of confusion in the healthcare industry on the exact bile acid level result that requires an official ICP diagnosis. There are different bile acid tests on the market, each with their own reference ranges, making things very confusing. She explains that the US healthcare professionals currently view bile acid over 10 µmol/L as an indicator of ICP; however, this may soon change to 14 µmol/L. “Some doctors don’t know that 10 is the level, and so a lot of people who have ICP are not being diagnosed. This is because some labs consider up to 19 µmol/L as being normal,” Dr Bonebrake says.

Dr Bonebrake shares that there is also some misinformation and confusion surrounding “fasting versus non-fasting”, where patients are told by labs to not eat. “There has been some data which shows that if you don’t eat, your level might not get to diagnostic levels,” she explains. This means some women are attending bile acid testing while fasting, and others are not, creating inconsistent results and uncertainty around what levels accurately warrant an ICP diagnosis.

“There is a lot of streamlining that needs to occur with the bile acid testing, that’s for sure.”

2. There is a lack of effective treatment options for the ICP community

Although treatment exists to help with some of the symptomology that sits with ICP, for example topical creams to stop itching and medication to help with insomnia, the current first-line treatment being prescribed to the ICP community to lower bile levels is not successful for all women. 

Laura feels fortunate that she did somewhat respond to this first-line treatment in her first two pregnancies; however, she responded less well in her third, more challenging pregnancy. “I don’t know why I lucked out by responding to the treatment the way I did, but for many women it doesn’t help at all. Results are very inconsistent.” Laura explains that this treatment has also not been proven to decrease the risk of stillbirth. “Even though the medication lowered my bile acid levels, we couldn’t say it would lower the risk of stillbirth because we don’t know exactly what causes the stillbirths to happen. That was the hardest part for me because myself and my husband just wanted that guarantee, and unfortunately, it’s not there.”

Dr Bonebrake says that national and international clinical trials are currently exploring new treatment options to help lower bile acid levels further and investigating if this could protect the unborn child. One of these trials, currently enrolling patients in the UK and US, is studying a novel drug that may offer a new treatment for ICP patients, and ICP care has advised the pharmaceutical team concerned.

“I am hopeful that these clinical trials may show promise. It would be nice to have a guarantee of safety for ICP moms in the future.”

3. There is new guidance on delivering a cholestasis child

“Because of the risk of stillbirth, ICP moms deliver prematurely. I delivered my children anywhere between 35 and 37 weeks, depending on what my bile acid levels were.” Laura explains it can be difficult finding the best time for ICP mothers to have their children. 

To decrease the risk of stillbirth, it is often important to deliver the pregnancy prematurely, but there are also risks that come with having a premature infant too. Dr Bonebrake explains that there have been debates for some time within the medical community, which has conflicting opinions on the delivery timeline for ICP mothers.

The current guidelines from American Congress of Obstetricians and Gynecologists (ACOG) and Society for Maternal-Fetal Medicine (SMFM) recommend delivery between 36 0/7–39 0/7, to be considered on an individual basis. If levels are over 40, delivery should take place at the earliest point in this window. If bile acids are greater than 100, delivery should occur at 36 0/7 weeks with consideration for earlier delivery in extenuating circumstances.

The upcoming launch of an ICP patient registry

Aside from raising awareness on the important issues and topics covered in this article and more, which you can read about on the ICP Care website, Laura and the rest of the ICP Care team have been working hard on another project for the ICP community: the upcoming launch of their ICP patient registry.

“We want this patient registry to give moms a voice, and hopefully gather a database large enough that we can better understand the disease and answer the questions that we are unable to currently.” 

Dr Bonebrake would like the patient registry to also provide insights on the emotional burdens the ICP community faces. “We are starting to see more conversations happening on the emotional side of ICP but talking about stillbirths is still almost a taboo topic.” She would like to see more awareness on the trauma ICP can cause families, and see more support implemented to help them during and after pregnancy. 

“It is exciting to see improved guidelines and better awareness of ICP recently. Although there is still a need for more research, we are hopeful that the momentum will continue to help ICP patients.”

Resources

ICP care website: icpcare.org

ICP Care healthcare provider micro-learning video: youtube.com/watch?v=cYhexEvcJeo

Photo credits

Portrait of Dr Laura Bonebrake: Jennifer Mossman Photography

Family photos: Cari Lane Photography

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