Miller Queen suffers from a severe and debilitating form of epilepsy known as Dravet syndrome, but you’d never know it by looking at him. At 7 years old, Miller is doing things that never seemed possible like playing soccer and going to school.

“Daily life when you don’t have seizure control is just a rollercoaster,” his mother Chelsea Queen told NBC 5. “You’re kind of always on edge waiting on the next seizure to happen.”

Starting at 6 months old, Miller was having as many as 10 seizures a day. Every treatment he tried failed and his parents were afraid to take their eyes off of him for even a moment.

Then, they found Cook Children’s and M. Scott Perry, M.D.,  an epileptologist and medical director of Neurology and the Genetic Epilepsy Clinic at Cook Children's.

“When you deal with epilepsies that are so difficult to control, it certainly becomes difficult not to feel that there’s not an answer,” Dr. Perry said.

Miller was enrolled in a clinical trial for the drug fenfluramine, which was once used as a popular appetite suppressant. Only a handful of hospitals nationwide were involved in the trial and Cook Children’s happened to be the only one in Texas. The drug completely stopped Miller’s seizures.

“The treatment completely changed his life,” Queen said in the interview with NBC 5. “He’s always been such a happy kid but now he’s able to experience life to the fullest.”

Overall, the clinical trial showed fenfluramine reduced seizures on average by about 70%. The research helped lead to approval from the Federal Drug Administration, opening up fenfluramine as a treatment for everyone with Dravet syndrome.

“The next medication to try and make you seizure-free is probably 2%, but you know what, you might be that 2% so that’s why we keep looking for it,” Perry said.

For Miller’s parents, they know this to be true.

Matt Queen, Miller’s father told NBC 5 “If something doesn’t work, don’t lose hope.”

“All of these children presented to the hospital with symptoms that resembled a severe case of Kawasaki disease,” said Nicholas Rister, M.D., an infectious diseases physician at Cook Children’s.

Kawasaki disease is an illness that creates inflammation in blood vessels with no proven cause, but is generally thought to follow various infections after they have otherwise resolved. Dr. Rister said the patients with MIS-C arrived at Cook Children’s following exposure to COVID-19 and had symptoms including fever, abdominal pain and outward evidence of inflammation including diffuse rashes, conjunctivitis and swelling. In the more severe cases, evidence of multi-organ dysfunction including respiratory distress, low blood pressures, liver and kidney damage and altered mental status were also seen.

“Of particular concern to us is inflammation of the heart and surrounding major blood vessels which is also seen in Kawasaki’s disease. We have seen this same thing in several of these COVID inflammatory disease patients,” explained Dr. Rister. “Minimizing the degree of inflammation in these children, while providing supportive care for any organ damage, has been a key component of treatment.”

All four patients were tested for COVID-19. Three tests came back negative and one was positive.

“We believe all of these cases are related to COVID-19,” said Dr. Rister. “The three negative results are evidence of how far the infection had progressed, resulting in the inflammatory syndrome.”

Three of the patients have been released from the hospital. One remains in the pediatric intensive care unit.

In addition to the recent appearance of MIS-C cases, the infectious diseases team at Cook Children’s is also looking closely at increased reports of unexplained fevers in our area.

“We want parents to be aware of these cases as COVID-19 continues to spread in our community,” said Dr. Rister. “Unexplained fevers for several days and evidence of generalized inflammation may be signs of this illness.”

Symptoms of more severe MIS-C cases include severe abdominal pain, shock from low blood pressure, respiratory distress and lethargy. If your child exhibits any of these symptoms, seek emergency medical care immediately.

Less severe symptoms include fever, abdominal pain and rash. Caregivers should call their pediatrician if these symptoms appear, as they overlap with many other common infections and medical conditions. It is important for these children to be fully evaluated.

COVID-19 has now been associated with a syndrome in children known as pediatric multi-system inflammatory syndrome. This condition is similar in nature to two pediatric conditions known as Kawasaki disease and Toxic Shock Syndrome.

While we still don’t know a lot about the COVID-19 related syndrome, let’s take a look at Kawasaki disease.

Kawasaki disease is a syndrome in children which is characterized by fever over 5 days with some of the following findings:

• Swelling or redness of the hands and feet, followed by peeling of the tips of the toes or fingers
• Red rash all over
• Redness and crusting of the lips or redness of the tongue (like a strawberry)
• Severe pink eye without drainage or mattering
• Swollen lymph nodes of the neck

In addition, children are commonly very irritable with Kawasaki disease. It is significant because the level of inflammation can affect multiple organs (most notably the heart) and lead to very severe illness.

While it’s still not a common condition, even with the newfound association with COVID-19, parents should be aware if their children display persistent fever with any of the associated symptoms.

The cause of Kawasaki disease is unknown. Some evidence suggests there might be an infection involved. The symptoms and complications associated with it are a result of inflammation of small to medium sized arteries throughout the body.

The most commonly known complications of Kawasaki disease involve the heart and can be inflammation of the lining or muscles of the heart, or dilation of the arteries that supply the heart.Toxic Shock Syndrome can present in the same way as Kawasaki Disease but the patient has low blood pressure and is significantly more ill.

If you suspect your child has Kawaski disease or Toxic Shock Syndrome it is important to seek your doctor’s advice quickly, as it is much easier for your physician to connect the dots when symptoms are present. Blood tests can help support the diagnosis and treatment can resolve the symptoms and help to prevent complications.

The good news is that children with Kawasaki's disease and Toxic Shock Syndrome, usually return to normal and don't suffer complications.

For more on this topic:

• Kawasaki disease (cookchildrens.org)
• Pediatric multi-system inflammatory syndrome
• COVID-19 and Pediatric Multi-System Inflammatory Syndrome (AAP)
• Kawasaki disease
• Toxic Shock Syndrome
• My Child's Fever Isn't Going Away. Should I Be Worried?
• Chest Pain. Is It Your Child's Heart or Something Else?

The story of Rylie Whitten has recently made national headlines. Her doctors say she suffered one of the worst cases of toxic shock syndrome they had ever seen. For nearly two weeks Rylie was unconscious but she is currently off life support and giving doctors hope of recovery from this rare syndrome that affects only about 1 in 100,000 people.

Still, parents may want to uses Rylie’s story as a cautionary tale for their own daughter.

What is toxic shock syndrome?

Toxic shock syndrome (TSS) is a group of symptoms throughout the body. This illness can progress rapidly and lead to a failure of multiple body systems. Toxic shock syndrome can be fatal. It’s caused by two types of bacteria, Staphylococcus aureus (often called staph) and Streptococcus pyogenes (often called strep). These bacteria can produce toxins. In some people whose bodies can't fight these toxins, the immune system reacts.

What causes TSS?

Factors that can increase your risks of TSS include chickenpox, skin lesions such as burns and HIV infection.

But what’s mostly associated with the cause of TSS is tampon use. The earliest cases date back to the late 1970s and were caused by super-absorbent tampons. Research produced better, safer tampons and better habits, such as changing them more often, caused a significant decrease. Today, about half of all TSS cases are linked to menstruation.

Other causes include two types of birth control methods – the contraceptive sponge and the diaphragm.

What should your daughter do to decrease the risk of menstrual-associated TSS?

Your daughter should only use low absorbency tampons and change them often.

Risk of menstrual-associated TSS can be lowered with the following steps:

1. Do not use tampons continuously when menstruating.
2. Alternate using a tampon with a sanitary pad.
3. Switch to sanitary pads at night.
4. Do not use super absorbency tampons.
5. Change tampons frequently during the day (every 4-8 hours).
6. Store tampons in a clean, dry place.
7. Wash your hands with soap and water before and after using a tampon.
8. Use a lower absorbency tampon if you find the tampon is irritating or hard to remove.
9. Use tampons only during menstruation.
10. Seek medical care for infected wounds.
11. If you have had TSS, do not use tampons or place birth control devices in your vagina.

Resources:

• Toxic Shock Syndrome
• Toxic Shock Syndrome Information Sheet

*Photo from Spectrum Heath "Heath Beat"/ Courtesy of the Whitten family

“Until a couple years ago, no one could tell us what she had. Everyone said she was ‘developmentally delayed,” said Jill Gomez, Alyssa’s mom. “I never expected to have a diagnosis.”

Though she’s actually a teenager, Alyssa looks like she may be a four year old, and has the mental capacity of a toddler. She has an extremely rare genetic disorder known as Syndrome X, meaning she may never age.

“No one knows why this occurs, but there is research being done on cases like this, including Alyssa’s,” said Scott Perry, M.D., medical director of the Epilepsy Monitoring Unit and Genetic Epilepsy Clinic at Cook Children’s Medical Center. “She’s truly a special child. I’ve never seen another patient like her.”

Though Alyssa lives in Hobbs, New Mexico, she sees Dr. Perry twice a year for help controlling seizures which come along with her disorder. She also has problems with a swallowing dysfunction and recently underwent surgery to correct the issue. But her life is not all pokes and prods in hospital rooms. Jill and her husband Eli work to keep life as normal as possible for Alyssa and her three siblings.

“We’re just a normal family. We take it day by day,” said Gomez.

However, being one of of five or six children in the world with Syndrome X, Alyssa can’t help but have a unique story to tell. On Monday, July 18, her story will be told on national television in a new documentary called ‘Girls Who Don’t Age.’ Dr. Perry will also be featured on the show. It airs at 8pm (CST) on TLC.

“It’s exciting. We’re excited to see it,” said Jill. “I just hope it might help another family out there that may be struggling to find an answer about their child.”

Marc Mazade, an Infectious Disease specialist at Cook Children’s, first heard about Middle East Respiratory Syndrome (MERS) at a presentation last year.

While Dr. Mazade says the attack rate of MERS seems low compared to other respiratory illnesses, he does advise certain precautions if traveling overseas.

“Returning travelers who develop signs of illness should discuss with their healthcare provider their full travel itinerary, exposures they may have noted during travel, and activities in which they participated while traveling,” Dr. Mazade said. “For a physician, the key to a prompt diagnosis of any potentially serious disease like MERS is making the connection that a patient or family has traveled recently to the Arabian Peninsula or other area where an outbreak has been documented.”

The CDC reports “most MERS-CoV cases have been reported in adults (median age approximately 50 years, male predominance), although children and adults of all ages have been infected.” Pediatric Infectious Disease Journal said that severe disease can occur in children with underlying conditions.

First, Dr. Mazade suggests those who are traveling from the U.S. to other countries to always check the CDC website for health-related travel advisories before making travel plans. Six to eight weeks before visiting overseas, Dr. Mazade advises people to receive a travel clinic consultation from their physicians.For more information, read the CDC's tips on traveling smart.

Dr Mazade said a travel clinic consultation will help determine if travel vaccines or malaria prophylaxis are necessary, as well as provide:

• An opportunity to receive counselling about other health risks a traveler might face.
• Whether to buy travel insurance or not.
• How to register a travel itinerary with the State Department to get a replacement passport in the event that one is lost or stolen.
• What items to take and what to avoid. 

“We consider fever in a returning traveler to be a serious concern and encourage travelers to stay in touch with their physician about any health problems occurring after traveling,” Dr.  Mazade said. “Visiting other countries can be exciting. Staying healthy and being aware of risks when traveling will keep the memories pleasant.”

The Centers for Disease Control and Prevention has reported the first case in the United States of MERS to be transmitted from one person to another.

The CDC states, the first U.S. Case of MERS was confirmed on May 2, 2014, after someone traveled from Saudia Arabia to the U.S. On May 11, 2014, a second U.S. “imported case” occurred, again from Saudia Arabia. The third case came as a direct result of an Illinois resident who had contact with the first case.

According to the CDC, Middle East Respiratory Syndrome (MERS) is a viral respiratory illness first reported in Saudi Arabia in 2012. It is caused by a coronavirus called MERS-CoV. 

The CDC says MERS “represents a very low risk to the general public in this country,” but will continue to monitor the situation and provide recommendations as necessary. At this time, no vaccine against MERS exists.