Brooklyn Rex was diagnosed with Hemophagocytic Lymphohistiocytosis (HLH), an orphan disease often referred to as “the beast”. It is a cancer-like disease that affects the immune system, in that it causes it to become over active and starts attacking the organs and which can lead to multi organ failure. Not a lot is known about HLH as it’s a very rare disease.
Statistics show HLH affects 1 in every 1.2million children. However, successful diagnosis of the disease and greater awareness is proving the statistics to be more like 1 in every 500,000 children are affected.
There are two types of HLH – Primary (genetic) & Secondary. We were not sure which type Brooklyn had as there is no genetic testing in South Africa for HLH. The genetic type of HLH has a 100% mortality rate if not treated within two months.
HLH is treated like cancer with chemotherapy however since her HLH was suspected to be secondary we started with first line treatment which was 8 weeks of high dose steroids. Steroids suppress the immune system making you more predisposed to illness.
This requires us to stay in isolation and try keeping Brooklyn away from crowds of people especially children as she is not up to date with vaccinations. It is hard to sometimes explain to others how a common cold or flu could kill our child. This is especially hard because she loves children.
We noticed over the last couple of months that Brooklyn would battle to walk especially in the mornings. After monitoring her and doing lots of research we came across something called Systemic Juvenile Idiopathic Arthritis (SJIA).
SJIA can trigger a type of HLH called Macrophage Activation Syndrome (MAS). We contacted the Head of Paediatric Rheumatology at The Red Cross Children’s Hospital in Cape Town. With our first consultation with the doctor, Brooklyn was confirmed to have Systemic Juvenile Idiopathic Arthritis as we suspected. The doctor said Brooklyn’s arthritis was the worst he had seen in a child of her age.
After starting chemotherapy and a few more consultations we decided to start the biologic drug that was suggested to us. Anakinra (Kineret) is a section 21 drug that we now import from Switzerland for Brooklyn’s arthritis.
In the beginning stages of starting this drug Brooklyn landed in ICU twice, whilst in Cape Town, with a nasty bacterial infection in her port (installed to help with chemo treatment) leading us to remove it. Along with the Anakinra, Brooklyn has been put on Ciclosporin and continues with her steroids. We are currently weaning her off the steroids as they have long term side effects.
This process will take two months if all goes well. We will then wean her off of the Ciclosporin which can also have long term side effects. Brooklyn will hopefully maintain a healthy state on the Anakinra alone indefinitely.
These meds do not come without restrictions and requirements. Brooklyn’s Anakinra (Kineret) is a costly medication and averages R21 000 a month based on the exchange rate of the Euro. This is not covered by any South African medical aid because it is a Section 21 drug.
Ciclosporin is another expensive medication that costs R3500 for each bottle also not being covered by medical aid. Lastly, Brooklyn’s disease requires her diet to be complete so she has to drink a prescribed milk formula called Peptamen Junior. This milk is costly running in at just over R250 a tin that lasts two and a half days, costing just over R3000 per month – not covered by medical aid.
At this stage we would like to ask for assistance with the costs of Brooklyn’s biologic, Ciclosporin and specialised formula which is approximately R27500. Thank you.