Primary Immunodeficiency Disorder in children: Why this dad is running for his son...
Primary Immunodeficiency Disorder in children: "I want to go through the suffering that my son is going through..."
There is a heartwarming reason why this dad is running this year’s Standard Chartered Singapore Marathon.
He wants to show his son that nothing is impossible.
Adhi Nugraha's only child Razqa is only 2, but has been diagnosed with a rare disease known as Primary Immunodeficiency Disorder (PID). He is currently receiving treatment in Singapore.
Adhi tells theAsianparent more about this condition, and how it has affected his son and the family.
Do tell us a little bit about yourself and your family?
My name is Adhi Nugraha Sugiharto and I am currently working at Maybank Indonesia as a project manager in the Strategy & Innovation unit.
My wife is Nurul Hudayanti, manages her time between being a full time mum and a part-time legal executive.
Our son's name is Razqa Aakarshan Sugiharto. He was born on 26 January 2015. Razqa is our only son.
When did you first get to know about your son’s condition? What were the initial symptoms?
Razqa was first hospitalised in July 2015, when he was only 6 months old.
He had a very high fever (> 39°C). He was hospitalised for 10 days without the paediatrician even knowing the underlying cause, so we decided to get him out and see another paediatrician.
This doctor reviewed all of Razqa’s medical records and treated him with the right antibiotics. After 3 days, Razqa's condition significantly improved.
However he still suffered many recurring infections throughout the course of 3 months, from July – Oct 2015. Ear infections, swollen lymph node, and abscesses.
Razqa underwent his first surgery to remove his swollen lymph node in August 2015. After the surgery, he still faced many infections.
In early October 2015, our doctor delivered the bad news - that he suspected Razqa to have a Primary Immunodeficiency Disorder (PID) and recommend that we perform a comprehensive test to really know what type of PID he had.
His frequent and recurring infections, abscesses and the swollen lymph node were main indicators of someone who has PID.
We then decided to do an extensive research of doctors who specialised in immunology in Singapore. Our online research led us to KK Hospital.
From there, one of KKH’s senior consultants (forgot his name) recommended us to consult Dr. Liew Woei Kang in Gleneagles Hospital. Dr. Liew Woei Kang suspected that Razqa was suffering from a Chronic Granulomatous Disorder from the symptoms that he had in the past 8 months.
He recommended Razqa to have his blood tested (Neutrophil test) specifically to see whether his blood could fight infections caused by bacteria and fungus.
The test results clearly showed that Razqa has a deficiency consistent with CGD.
It was a very emotional day for us - our only son was diagnosed with some congenital disorder that could be fatal for him and would affect his childhood.
How is this condition normally treated?
After knowing my son’s condition, we immediately met our paediatrician in Indonesia with the test results. He recommended us to meet an immunologist to handle Razqa’s condition.
Coordinating with Dr. Liew in Singapore, Dr. Harun immediately prescribed Razqa with a prophylaxis anti-biotics and anti-fungal that Razqa has to take every day.
How common is Primary Immunodeficiency Disorder in children?
Based on research in a prominent medical journal, only 1 in every 250,000 newborns is affected with CGD. CGD is a very rare disease.
My wife and I have always questioned why Razqa had to be the one. However, we soon realised that we need to empathise with all patients affected with CGD or PID in general.
The challenges, the ups, and downs of Razqa’s journey in the past 2 years have built our empathy for others and provided us with true lessons of the struggles in life.
Did you have to make any changes to your lifestyle?
From our side, as Razqa’s primary support system, we changed the way we treated Razqa drastically.
We understood that we needed to make Razqa’s environment clean, things like cleaning our house with disinfectant daily, installing a 24-hour air purifier, sterilizing his eating utensils, and many other preventive actions were taken.
We also understood that there are a few things that Razqa will have to miss out as a child.
As a CGD patient, Razqa could not swim in public swimming pools, play with soil in a garden or go to a dusty place. All this would expose him to organisms that were specifically dangerous in CGD.
Is the treatment expensive?
Monthly, almost 20% of our income is spent in managing Razqa’s medical condition (medicine, routine consultation, and follow-up test).
We are also investing a significant amount of our income for Razqa’s insurance - having a child with CGD made us realise the importance of having insurance for financial protection.
As of now, we are looking for a permanent solution to his PID, where one of the options is to do a Stem Cell Transplant that is not available in Jakarta. The cost of a Stem Cell Transplant can range between SGD 250,000 – SGD 300,000.
Hoping for a miracle, we have registered Razqa for a Stem Cell Donor search process in KK Hospital. Should a miracle happen and Razqa gets a perfect match for a donor, we would do everything within our means to provide for his Stem Cell Transplant process.
We are even prepared to sell our own house.
How did you decide to participate in the Standard Chartered Singapore Marathon (SCSM)?
I want Razqa to see that nothing is impossible. If it is possible for me (a non-athlete) to complete a 42.1 km race, anything is possible. I also want to inspire him to see that anything is possible if you put your mind into it.
Discipline, something that is shown in my process of training for the race, will be a valuable lesson for him. Lastly, I want to go through the suffering that he is going through.
I consider running a marathon and the training journey an extremely taxing one. Pain is something that he is very accustomed to and it is something I want to experience as well, to let him know he is not alone in suffering.
I have always been into running and an active lifestyle. Working in Maybank, I also have the responsibilities of managing the Maybank Bali Marathon as one of the project managers.
From an early age, despite his limitations, I have introduced Razqa to running. His first race was the Maybank Bali Marathon 2016 children sprint category, where he ran for 400 m.
Last August, he once again participated in the Maybank Bali Marathon children sprint. I want him to be aware of the benefits of having a healthy lifestyle and most importantly I want him to feel that there is no limit to what he can do if he put his mind into it.
And then, there is a more important reason for taking part in this marathon. As parents of a kid with PID, we find that we are very lucky to have our son diagnosed early so that we can manage it this early in his life.
This ensures that he can be relatively well and get through many potentially fatal infections.
That is why in January 2017, we officially joined the Indonesia Primary Immunodeficiency Patients Society (IPIPS). The society aims to give awareness and acts as a support group for PID patients to meet and support each other.
I am very humbled to be assigned as an interim president to the group. My wife and I see this as an opportunity to give back as we are lucky enough to have our son diagnosed and treated early on.
Currently, the group is in progress of preparing an online awareness campaign for PID. That is why for SCSM 2017, I would like to promote awareness of Primary Immunodeficiency Disorder in children simply by wearing a shirt that can attract runners to read and also learn more about PID.
What is your advice for other parents whose children suffer from similar conditions?
The first step is to be aware of what Primary Immunodeficiency Disease in children is, and what are the symptoms.
The Jeffrey Modell Foundation provides a simple 10 warning signs information for parents to be aware with Primary Immunodeficiency.
Some of the warning signs for Primary Immunodeficiency Disorder in children:
- Children developing 4 or more ear infections in a year
- Recurrent deep skin abscesses
- Persistent thrush in the mouth or fungal infection in the skin
As a parent, we were very lucky to have Razqa diagnosed very early (8 months) as we could manage his condition better.
We also found that having a knowledgeable and committed paediatrician is very important for managing children with PID. We are very grateful to find a paediatrician who is very objective and very much aware of PID, as PID is still considered a rare disorder.
I cannot stress enough the importance of having a knowledgeable immunologist who can contribute more information for the well-being of your son/daughter.
Lastly, it is very important to have empathy for your children with PID. If it is tough for you to manage, you can imagine how tough it is for him to feel the pain/irregularities in his body all the time.
Always be positive in every step of his journey. There will always be rainbow after the rain.