Having a chronic illness comes with many challenges. The main goal is to stay healthy and live as normal a life as possible. The additional goal of a sickle cell patient is to have as few pain crises as possible.
You want to make sure you do nothing to trigger that pain. Eating a proper diet, managing stress, getting plenty of fluids, getting the proper rest and taking your meds are all key elements to a life with less pain.
Since the summer of 2020, Adakveo, an infusion medicine, has come to market and it’s a game-changer as far as managing sickle cell pain.
It is given once a month by an IV infusion and is meant to impede pain crisis. “It prevents the abnormal stickiness of the red blood cells from causing more pain,” explains Dr. Lewis L. Hsu, MD, PhD, Professor at University of Illinois at Chicago, Pediatric Hematologist. “The clinical trials of this drug were very promising in cutting down sickle cell pain episodes.”
One great advantage of getting an IV medication is that you take it once a month and you don’t have to worry about forgetting to take a daily pill. “If the patient’s weight changes, IV is a good way to adjust the dose because the patient is right there getting the IV and you can change it right there. Compare that to a medicine the patient takes at home – you’d have to figure out the dosing, tell the patient, prescribe it to the pharmacy and in hopes that everyone remembers and you got the right dosage,” continues Dr. Hsu. “[Adakveo] does not seem to require a monitoring of lab test to look for organ damage.
It does seem to need monitoring during the infusion to see what kind of infusion reaction there can be because this is an antibody type of medicine. Sometimes when you infuse an antibody product, the body reacts to it. That is very common to see side effects of those sorts and the infusion room staff will watch the patient closely.”
After the first couple of doses, the infusion will continue to be given monthly until it is determined whether a more or less frequent schedule is needed. Adakevo stays in the system without the drug diminishing completely by the end of the month.
Dr. Hsu explains, “Right now, we are just going to infuse it monthly. There have not been any studies of a schedule other than monthly, so we don’t know how much better it might be if it’s not given monthly.
The whole idea is that it’s supposed to be preventive. The effect is waning by 3 ½ weeks by maybe 50 percent, but it hasn’t completely gone away. The idea is to boost it back up and keep it at a good level.”
This infusion process is being monitored. If the track record of the infusions ever gets to a point where no one has reactions, it’s the hope that future infusions could eventually be given at home, but they are not there yet.
Right now, infusions are given at infusion centers where you are with a full medical team, resuscitation equipment, allergy reaction treatment all things available if needed.
Dialysis Centers are places that could be used to give the IV infusion because they attach IVs to patients, but a better fit, according to Dr. Hsu, are places where chemotherapy or infusions of other antibody medicines are given. “A lot of diseases have these antibody type medicines, some GI [gastrointestinal] diseases like IBD [inflammatory bowel disease], some arthritis type conditions and some immune conditions. They all have similar kinds of treatments that are given once a month or once every two months with an IV infusion and you have to watch for a reaction and allergic problems.”
Most cancer centers have infusion rooms. At the University of Illinois-Chicago where Dr. Hsu practices, there is one for adults and one for pediatrics.
Going to these types of places is easier than going to an emergency room. But now, there are some places that specialize in pain treatment. Instead of the emergency room or other places, you go to a place that specializes in sickle pain: sickle cell pain centers or sickle acute care centers.
At these centers, everyone on the staff knows what to do for sickle cell straightforward pain, and they also know how to recognize other sickle cell problems (like acute chest syndrome) that require more than just pain medications. It’s an expert disease-focused center.
There are very few of these pain centers. Chicago only has one for adults at the University of Illinois-Chicago. Most states have none. “People travel a considerable way to get there, but we get as fast a pain treatment as we can do. Still not as fast as we would like, but there are still barriers of the check-in process, and COVID testing, getting an IV and things like that, but we would have familiarity with this person, we know their usual treatment plans, be able to get labs results swiftly and be comfortable with how to watch for other sickle cell problems. So, these [sickle cell pain centers] are able to give people an alternative to going to this hustle-bustle emergency room. We hope this is part of the new definition for what a sickle cell center is,” states Dr. Hsu.