A mother has described her shock after her daughter's rash, initially thought to be weather-related, turned out to be a life-threatening condition. Starr Downs first noticed the rash on her daughter Esmae Smith's hands and feet on April 6 this year, but assumed it was an allergic reaction or due to the weather.
Rash dismissed as hives
Taking Esmae to the pharmacist, the 23-year-old was told it looked like hives and was prescribed anti-itch cream until she could get a doctor's appointment. Starr claims Esmae's temperature kept spiking to 40 degrees, and by the time she saw a doctor on April 8, she was struggling to walk up stairs.
The pair were sent to hospital, where Esmae was kept for eight days as she went 'downhill really quickly' but doctors remained unsure what was wrong.
Diagnosis at Great Ormond Street
Esmae was transferred to Great Ormond Street on April 16, where she received the diagnosis of Macrophage Activation Syndrome (MAS). The mum says doctors explained the disease by comparing the youngster's white blood cells to the iconic computer game Pac-Man, in which the character 'gobbles' up cells for points.
However, rather than just targeting infections, the youngster's cells are 'attacking her immune system' and 'eating everything they see', in a way likened to the 80s hit video game. Starr has now set up a GoFundMe to fund travel costs and to give Esmae a 'big treat' once she is feeling better, as she is now undergoing gruelling chemotherapy treatment.
Mother's fear
Starr, from Southend-on-Sea, Essex, said: "To start with I was just thinking it's probably an allergic reaction or like weather change or something. For something so minor to end so big, it's really scary."
"It started with a rash on her hands and her feet and she was sore around her mouth and one of her eyes was bloodshot. I thought just an allergic reaction, I just thought like a rash, I don't know where she would've got it from."
"I took her to the pharmacy and they said it looked like hives and they gave us some itch cream relief until we could get a doctor's appointment. She couldn't really walk, she was limping. We went to the doctors and there's stairs and every step we went up to get to the doctors she just moaned that it hurt."
"We stayed in hospital for eight days and they kept saying to us they're not sure what it is. We [were transferred] to Great Ormond Street and then two days later is when the rash came really bad."
"They said to us it's life-threatening and that she was going downhill really quickly and children don't normally deteriorate that badly. I was so scared, she wasn't responding, she was just laying there just staring into space."
"This is how they explained it to me: in our bodies we have blood cells that fight infections. They go around the body like Pac-Man and they eat the infections, but Esmae's ones, they don't know when to turn off so they're just continuously eating everything they see. They're not just attacking the infection, they're attacking her immune system, so her liver is damaged and her spleen is swollen."
Cause and treatment
Starr claims they are unsure what has caused Esmae to become unwell, but that doctors currently believe the MAS has been triggered by Systemic Juvenile Idiopathic Arthritis (sJIA).
Starr said: "I just broke down and started crying, I couldn't sleep because I didn't realise how severe the problem was. [It's] really scary because I'm so young and she is my only baby and I know age doesn't matter, but I do feel worse because I'm young."
"She was never poorly she was generally just a fit and healthy child, doing normal kid stuff and then all of a sudden everything just went downhill. We had no choice but to start the chemo drugs because she's deteriorating really badly and it is life-threatening. It did bring her back around."
Doctors have said they are unsure when Esmae will be able to go home, but Starr will have to give Esmae immunosuppressant injections daily once she is out of the hospital.
Starr said: "It's really scary because if she is to get an infection in the future, will her body be able to fight it. I know we're in the safest of hands and obviously she has come a long way. I just believe that Esmae deserves a big treat after."
Macrophage Activation Syndrome (MAS) is described by the Arthritis Foundation as a massive inflammatory response that takes over the whole body. What triggers the body's overactive immune system in MAS is unclear, but it is thought to be a reaction to infection in about half of cases.
MAS symptoms include a high, constant fever, changes in alertness, easy bruising and an enlarged liver. Treatment for MAS involves trying to reduce body-wide inflammation as quickly as possible to prevent organ damage and chemotherapy drugs can be used if MAS continues.
