For 81-year-old Abdul Fazal from Bengaluru, life was tough for almost a year when he could not bite into food. Doctors had to remove his upper jaw while treating a condition that came upon him – mucormycosis. Mucormycosis is a fungal infection that can be caused by a group of moulds called mucoromycetes that can spread across the nose, eyes, lungs and brain. It can affect the functioning of the organs and gradually lead to cell death. Surgical debridement (removing the infected area) is the critical component of the treatment along with anti-fungal treatment.
Fazal lost his upper jaw and survived because fortunately, the infection hadn’t spread to his brain or lungs. Fazal is one of the many covid survivors who was parallelly infected with mucormycosis fungal infection during the second wave of the pandemic in India. Doctors term it a post-covid complication seen after the delta-driven second wave. While it was also called ‘black fungus’ because of the black discolouration seen in the infection spot, doctors now say that it was a misnomer.
Mucormycosis is angioinvasive – that means it penetrates the blood vessels, causing clots, says Dr Sujata Rathod, director, Minto Regional Institute of Ophthalmology, Bengaluru and director of medical education in Karnataka. The necrotic (dead) tissues become gangrenous and the fungus feeds on them. While the infection is seen in the nose for some, it spreads to the eyes for some. In a few cases, it also infects the brain and the lungs. “The fungal spores enter the nasal mucosa and further spread towards the eye, damage the optic nerve in the eyes and move towards the brain,” says Dr Sujata whose team treated hundreds of cases in 2021.
According to Dr Gaurav Chaturvedy, director, department of ENT and head and neck surgery, Jaslok Hospital, Mumbai, the symptoms of mucormycosis depend on where in the body the fungus is growing. Generally, mucormycosis affects the body surface and internal organs such as the sinus, brain, lungs, eyes, bones, nerves and body tissues – it can be fatal if left untreated. He lists out the symptoms:
- Facial pain, pain over sinuses, pain in teeth and gums
- Paraesthesia/decreased sensation over half of the face
- Blackish discolouration of the skin over nasolabial groove/alae nasi
- Nasal crusting and nasal discharge which could be blackish or blood-tinged
- Conjunctival injection or chemosis (eye redness)
- Periorbital swelling (swelling around the eyes)
- Blurring of vision or double vision
- Loosening of teeth, discolouration of the palate, gangrenous inferior turbinates (bony structures in the nose)
- Worsening of respiratory symptoms, haemoptysis or coughing up blood, chest pain, alteration of consciousness, headache
Dr Chaturvedy adds that mucormycosis is a rare but deadly disease with a 46 to 96 per cent mortality rate depending on the underlying health condition.
How does mucormycosis spread?
“This fungus is found in the environment. Transmission occurs through inhalation or ingestion of spores from the environment,” says Dr Chaturvedy. He adds that mucormycosis is an opportunistic pathogen that affects immune-compromised patients due to comorbidities, excessive administration of steroids, organ transplantation, exposure to ventilation, oxygen therapy and poor hospital hygiene.
Dr Rathod explains that mucormycosis cases involving the eyes were seen earlier too, but it appeared at an alarming rate during the pandemic. Government-run Minto hospital in Bengaluru witnessed a deluge of persons infected with mucormycosis in 2021 with blurred vision and discolouration around the eyes.
“We used to see not more than four to five cases of mucormycosis at a tertiary institute like Minto every year, but during the pandemic, we had up to 12 cases in a day. What was once a rarity became an everyday scenario. The challenge was that macrocytosis treatment required multidisciplinary treatment and it was not just about saving the vision,” Dr Rathod tells Happiest Health.
And what led to the sudden surge of the infection during the delta-driven second wave of the pandemic has remained a mystery, says Dr Rathod.
“Infected persons would initially have foul-smelling discharge and a feeling of obstruction in the nose. Those who came to us with blurred vision initially thought it was just a problem with the eyes,” recalls Dr Rathod.
She adds that the fungus enters the eyes and the optic nerve gets damaged as a result. The infection is also seen in the extraocular muscles of the eyes. “The optic nerve is an extension of the brain tissue. The optic nerve does not have any regenerative power and hence the condition is not reversible,” she adds.
However, most of those who survived mucormycosis infection is still under treatment for the reconstruction of their facial parts including the jaws, cheeks and facial bones.
“The common factors among the majority of the mucormycosis-infected persons were that there were highly diabetic, had a history of covid infection and used steroids during their covid treatment,” says Dr Girish Rao, honorary general secretary, Association of Oral and Maxillofacial Surgeons of India, who has been working on hundreds of mucormycosis survivors for the reconstruction surgeries.
Dr Rao adds that there is still a mystery that surrounds the surge in mucormycosis cases during the pandemic. “We don’t know if the covid cases in the second wave in India were overtreated by pumping steroids and if that’s what caused this. There are various questions yet to be answered,” says Dr Girish. This double-whammy was seen largely in India and not to such an extent abroad.
When a person is infected with mucormycosis, doctors have to debride or remove the entire affected area and put the people on anti-fungal medication, to prevent its further spread to the lungs and the brain as a life-saving measure. The anti-fungal treatment is for a tune of six to eight weeks (it varies depending on the severity). That’s the first phase of treatment.
Dr Chaturvedy adds that liposomal amphotericin B, an antifungal medicine, is the drug of choice and it needs to be initiated early. “Other antifungals like Posaconazole or isavuconazolehave have also been prescribed for treatment,” he says.
However, Dr Rao, mentions that after surgical debridement, people face a lot of facial deformities. “In some cases, the entire upper jaw, lower jaws, entire eyes, portions of the nose and cheek are removed and this affects their quality of life. People’s speech and swallowing are also hampered. A crippled face has a huge psychological impact,” says Dr Rao.
Facial reconstruction after mucormycosis
For some time, maxillofacial surgeons did simple removable prosthesis. “However that’s not efficient in terms of function and people can’t chew properly. Now we are using a 3D printed prosthesis with digital facial reconstruction. We reconstruct eye sockets, jaws and cheeks. Reconstruction is done only after the infection has completely subsided and the person is stable; it can take six months to one year. Reconstruction takes nearly six months, depending on the case. The reconstruction helps people to get back to normalcy with better speech and ability to chew,” adds Dr Rao
Dr Rathod points out that there is a question that remains unanswered – how did people catch the infection in the first place? “That has remained mysterious so far. There are no clear answers as to whether they were exposed to the fungal spores while being on oxygen support or whether it was the diabetes and the steroids that caused it,” says Dr Rathod. But he adds that the cases of mucormycosis gradually reduced by the end of 2021.
Back to life without the upper jaw
Fazal was diagnosed with mucormycosis during the treatment period. However, he was not diabetic and it may have been the steroids that made him vulnerable, suspect the family.
However, Fazal didn’t undergo reconstruction surgery. “Due to the age factor, we didn’t opt for it. He doesn’t have an upper jaw and he can’t bite. He takes masticated food. He is now doing his regular everyday activities, including driving. However, his appearance has changed and he is fine with it. He is not hesitant to leave the house anymore,” says his daughter Dr Tanzeem Ferdous. Fazal is off medication now.
According to Dr Chaturvedy, mucormycosis and its complications can be prevented if the following measures are followed:
- People with diabetes should be advised and encouraged to control their glucose levels
- Use steroids judiciously
- Rational use of antibiotics
- Timely initiation of amphotericin B therapy
- Keep a high index of suspicion in the presence of risk factors and do a daily examination of the eyes, nose and mouth to check for signs of infection
- Use clean sterile water for humidifiers
- Awareness and early diagnosis are the keys to halting the spread of the infection