Sickle cell disease is a painful and debilitating genetic blood disorder that has limited treatment options.

But a clinical trial for a new gene therapy that Cleveland Clinic is taking part in is showing promising results.

“We are seeing very encouraging results where the patients involved haven’t had a pain crisis from sickle cell after undergoing the gene therapy. Other colleagues who are participating in this clinical trial are seeing similar results for their patients as well,” explained Rabi Hanna, MD, the principal investigator of the study at Cleveland Clinic Children's.

Dr. Hanna said the trial involves an experimental gene editing cell therapy that modifies a patient’s own stem cells to correct the mutation responsible for sickle cell disease.

Two of the four patients involved in the multicenter trial so far were treated at Cleveland Clinic Children’s.

Along with relief from the pain sickle cell causes, initial results showed new white blood cells in all patients at about four weeks with no severe side effects.

They also achieved a normal level of hemoglobin, which is the most important component of red blood cells that carry oxygen throughout the body.

“Our hope is to achieve a functional cure. It wouldn't reverse the damage sickle cell disease has already caused. It would help prevent any future damage,” Dr. Hanna said.

The trial aims to enroll 40 adults – ages 18 to 50 – with severe sickle cell disease.

They will be monitored closely after treatment for up to two years.

These results were recently presented at the European Hematology Association Hybrid Congress in Frankfurt, Germany, on June 10, 2023.

“I really encourage parents to have set meal and snack times throughout the day. Snacks are important and kids can totally have snacks, but it needs to be spaced out every few hours, so their bellies have time to digest, and they have time to get hungry before the next meal,” explained Jennifer Hyland, RD, pediatric registered dietitian for Cleveland Clinic Children’s.

She said it’s also important for parents to offer snacks that are nutritionally dense.

Giving a child things like cookies or chips is not going to keep them full for long and they’re going to want to keep eating.

Instead, offer them foods high in protein and fiber.

Some snack ideas include an apple with peanut butter, smoothies, homemade popsicles, cheese and crackers, popcorn with peanuts, and whole grain cereal with milk.

She said it’s still okay to let them have cookies and chips, but in moderation.

“All within reason. If everyone is going out and getting ice cream, have your kid go out and get ice cream. But if it’s just a regular day and we’re hanging at home and doing our regular activities, we need to have a more nutrient power-packed snack,” said Hyland.

Hyland said another benefit to giving kids healthy snacks at an early age is it’ll be easier for them to adapt and carry on those habits as they get older.

Research from Cleveland Clinic has found that initial memory problems are linked with a slower rate of decline in Alzheimer’s disease and dementia. 

“What we found out was that people with early memory symptoms alone compared to people with early language problems or visual-spatial problems, they tend to have a slightly slower disease progression in terms of their functional abilities,” explained Jagan Pillai, MD, neurologist for Cleveland Clinic and lead author on the study.

Dr. Pillai said those with language problems, spatial ability and judgment issues seemed to be affected more over a period of time – specifically in their daily functional abilities. 

He said it seems to make sense since a person with just memory difficulties could find other ways to compensate, like keeping track of what they’re doing by recording themselves or writing down notes. 

That would then allow them to remain functional. 

He also noted that memory difficulties come from another part of the brain than language problems, for example. So, how the disease progresses could be different. 

“The portion of the brain that is first impacted by the disease tends to determine the symptoms that goes along with the disease and also the kind of progression from there to other regions,” said Dr. Pillai.

Dr. Pillai said having this information available can better help physicians diagnose people and prepare them for the future. 

It can also be useful for researchers when it comes to testing different medications to treat Alzheimer’s disease.

Now, Cleveland Clinic research has uncovered a possible culprit.

“The way we use our carbohydrates to make our energy; the way we use proteins and amino acids from our diet and other exposures, it was really a very strong relationship with cancer incidence,” said Suneel Kamath, MD, gastrointestinal oncologist at Cleveland Clinic and senior author of the research. “It's really something that's not been described before in colorectal cancer at all.” 

Dr. Kamath and his team studied samples of metabolites, which are substances created when the body breaks down food.

Results show people younger than age 50 with colorectal cancer had lower levels of citrate, compared to older people with colorectal cancer. Citrate is created when the body converts food into energy.

They also found important differences in protein and carbohydrate break-down, which could suggest that red meat and sugar intake may be related to developing colorectal cancer at a younger age.

Dr. Kamath said more research is needed, but there’s enough evidence to suggest it’s a good idea to reduce red meat and sugar in your diet.

“Certainly, limiting red meat consumption for sure. We've known that as a risk factor for colorectal cancer for a long time. And so, I would certainly say our data supports that,” Dr. Kamath explained. “Additionally, we know that obesity is a big risk factor for developing any cancer, including colorectal cancer. So, we think that the findings we showed with carbohydrate and energy metabolism probably relate to that to some degree as well.”

If you are experiencing symptoms of colorectal cancer like bleeding, a change in bowel habits or abdominal pain, Dr. Kamath said it’s important to reach out to your medical provider, regardless of age.

Study results were recently presented at the American Society of Clinical Oncology meeting on June 3.

“Nine out of ten individuals who have cardiac arrest actually die, and that’s because we can’t get to them fast enough to provide adequate CPR or defibrillator in a timely fashion,” explained Tamanna Singh, MD, cardiologist for Cleveland Clinic.

Dr. Singh said for those unfamiliar, CPR is a very simple process.
The first step is to make sure the scene is safe and call 911 or send someone for help, if possible.

Next, check the person’s vitals. If they are unresponsive and not breathing, it’s time to perform CPR.

They should be lying flat on their back.

You’re then going to put one hand over the other on the middle of their chest and start doing compressions 100 to 120 times per minute. Be sure to push down two inches every time.

Dr. Singh said you don’t have to worry about mouth-to-mouth breathing. Research has shown it is no longer necessary.

Besides CPR, it’s also important to know how to use an AED, or automatic external defibrillator.

The device can be found in many public places, like schools, stores and offices.

“You just turn it on and the device actually tells you exactly what to do, which is basically placing pads onto the person’s chest,” said Dr. Singh. “The device will identify whether the rhythm the patient has is one where an electrical shock is one that can actually revive them or make them responsive again. And if that’s really indeed the case, all you have to do is press a specific button on the device to do so.”

Dr. Singh said both adults and children should know how to do CPR.

There are many classes available both in person and online.

According to research, those who quit and go on to adopt a healthy lifestyle can reduce their risk of death.

“It shows that it’s not just about quitting smoking. It’s really about making these amazing lifestyle changes that will carry you through and really will lower your cardiovascular risk, cardiovascular mortality and death from other things such as cancers and respiratory diseases as well,” said Neha Vyas, MD, family medicine physician for Cleveland Clinic, who did not take part in the study.

The researchers found that former smokers who were stricter with their body weight, diet, physical activity and alcohol intake had a lower risk for death, cancer and other diseases. And that was regardless of previous smoking patterns.

Dr. Vyas said people may need to try multiple times before successfully quitting smoking.

However, she encourages smokers to not give up.

She’ll often tell people to avoid any triggering environments where there will be smoking, like at a bar or party.

It can also be helpful to build a support system, so you’re held accountable.

“It’s important to remember that it can sometimes take more than one attempt to quit smoking altogether, so certainly get support from your family, your friends, healthcare professionals. There are many resources out there,” she said.

The CDC reports that tobacco is the leading cause of preventable disease, disability and death in the United States. Nearly 40 million adults still smoke cigarettes.

It’s meant to raise awareness about the dangers of sunburns, ultra-violet radiation and overexposure to the sun.

Shilpi Khetarpal, MD, a dermatologist with Cleveland Clinic, reminds us that sunburn can cause skin cancer and some people are at higher risk than others.

“Those with fair skin are most likely to develop skin cancer in their lifetime,” she said. “So, if you have red or blonde hair, light eyes, are someone that freckles easily, if you’ve had sunburn in your lifetime -- those are all risk factors that will contribute to how likely you are to develop skin cancer.”

To avoid sunburn, check the UV index before venturing outside and avoid being out between 10 a.m. and 4 p.m., when the sun is at its hottest.

When outdoors, wear sun protective clothing, a hat and a good sunscreen.
A broad-spectrum sunscreen of 30 SPF or higher is recommended and one ounce is needed to cover the entire body.

Sunscreen should be applied 30 minutes prior to going out and every two hours afterward.

And remember, you can even get sunburned through windows or on a cloudy day.

A typical sunburn may feel warm, itchy or tender to the touch. It should flake or peel within a few days and heal in about a week.

“The skin is going to be very fragile because you’ve damaged a layer of skin,” explained Dr. Khetarpal. “So, make sure you avoid anything irritating, like anti-aging or anti-acne products. You really want to be gentle, so a gentle cleanser; and try and rebuild that barrier.”

Sunburns can be treated with aloe vera gel or petroleum jelly.

Acetaminophen or ibuprofen can also be taken for discomfort.

If the sunburn blisters on a large portion of skin, Dr. Khetarpal recommends seeing a dermatologist for additional care.

Tom Waters, MD, an emergency department physician with Cleveland Clinic said it’s a good idea to make sure your grill is working properly.

“When you’re going to light your grill for the first time, whether it’s propane or even charcoal, obviously you want to be careful,” he said. “If you aren’t able to light a gas grill after the first two attempts, you need to turn it off, let it air out and then inspect why it might not be working before you continue to try and light it to avoid any flare ups or big explosions of gas.”

Dr. Waters suggests checking your grill for gas leaks. He also recommends keeping a fire extinguisher handy.

You’ll also want to avoid wearing long-sleeved shirts while cooking so your clothes don’t catch fire.

A pair of heat-resistant gloves is a good idea as well.

If using lighter fluid, be sure to move it farther away once the grill is lit.

If you suffer a minor burn while grilling, it can be treated with cooling or antibiotic ointments.

However, if the burn is larger than the palm of your hand, you should seek medical help.

When it comes to fire pits or bonfires, make sure there is enough space between the flames and your home.

“You must watch what you’re burning and where the wind is blowing,” warned Dr. Waters. “If there are a lot of embers and they’re blowing towards a structure, whether it’s your house, your neighbor’s house, your kids’ playhouse, you need to monitor that because, sometimes, those hot embers could result in a fire somewhere else.”

Finally, Dr. Waters cautions never leave a fire unattended and be sure to put out the flames when you’re ready to go in.