Back in July, when I was dealing with a sick 9 month baby, I had never heard of neutrophils. Three months on, my learner plates have come off, so to speak, as I’ve learned a whole lot more about them.
I’m currently coming to terms with the fact that, Kaydes has got a rare blood disorder called Severe Chronic Neutropenia (SCN). Which is also a rare type of primary immunodeficiency. And what I thought may be short lasting, could actually be long lasting.. for how long I don’t know. It could be a lifelong thing for her. Tests are going to be run over the next couple of months.
A bit of backstory
After battling for weeks in June/July with viral Bronchiolitis, Kaydes’ poor body couldn’t fight it off very well. Due to its respiratory nature, I couldn’t take her to the GP, so I took her to Alice Springs hospital for a couple of visits.
By 31st July, she had developed Mastoiditis of her left ear. She was admitted in, and was to start IV antibiotics. She proved to be very hard to get a cannula in for those antibiotics to start, and to get bloods drawn. That alone was quite traumatic for the two of us.
My mum is a neonatal nurse at Alice Springs Hospital, so in and around her night duty shift she came and saw how we were doing. The next day with the doctors making their rounds, she enquired about Kaydes’ blood test results. That’s when I heard more about her neutrophils count. Her haemoglobin was low, and her neutrophils were severely low.
A neutrophil count is a measure of the number of neutrophils in a blood sample. Neutrophils are white blood cells that play an important role in the immune system, by fighting infections. A low neutrophil count can indicate a problem with the immune system. Neutropenia can be categorised by the following characteristics:
Severity – Based on ANC (Absolute Neutrophil Count) blood test:
- Mild: ANC above 1000 cells per microliter
- Moderate: ANC 500 cells per microliter to 1000 cells per microliter
- Severe: ANC < 500 cells per microliter (0.5 x 109/l)
Duration
- Acute: temporary or short-lasting
- Chronic: long-lasting neutropenia symptoms for more than 3 months
The doctors ordered a CT scan to see if the ear infection had spread any further. Fortunately it hadn’t, but it did require a trip to Adelaide Women’s and Children’s Hospital, to get surgery on it.
The surgery on 2nd August went reasonably ok. The mastoiditis portion where they drained the abcess in her left mastoid bone, and put in bilateral grommets went great, and took all of 10 minutes. She gave them a bit of a scare with her response to the GA though, where she had a laryngospasm.
She came round in the recovery room crying and scared, but otherwise well.
Neutropeni-huh?!
The next day after the surgery, some doctors made their rounds. And that’s when I heard more about neutropenia.
They said that the surgery on her ear went perfectly, and in fact her ear was already clearing up. But that her neutrophils are severely low, and she has neutropenia, and is immunocompromised.
They thought that the neutropenia will probably go away by itself, as it could be a response to the virus, or a counter effect on the antibiotics. We were discharged, and flown back home to Alice Springs, with follow up bloods to be done every few weeks.
This brings us up to now (November)
After recently turning 1 year old, and months of blood tests, with consistently low neutrophils scores of 0.3-0.4. Kaydes has now been classed with Severe Chronic Neutropenia (SCN), but with unknown cause right now.
Severe Chronic Neutropenia (SCN) is a bone marrow disorder that prevents your marrow from making enough neutrophils. Bone marrow is the tissue in the center of your bones that makes blood cells. Neutrophils are an important part of the immune system, and their main function is to protect the body against infection.
The two ways that neutropenia can develop are due either to the destruction of existing cells faster than they’re replaced, or if production in your bone marrow is reduced.
People with SCN are at a greatly increased risk for developing serious infections. As their bodies are unable to fight off bacteria and viruses effectively, which can be life-threatening. Treatment for SCN typically involves medication and/or bone marrow transplants. With treatment, most people with SCN are able to live long, healthy lives.
Because of the unknown origin, the next steps are more tests. We met up with her paediatrics specialist, who will be in touch with Adelaide WCH Haematology team, to see what tests they want done.
They most likely will want a genetics test, and/or bone marrow biopsy to see if it’s congenital (from birth), autoimmune or idiopathic (unknown cause). From there, we should hopefully know more, and what the treatment path looks like.
Hopefully, scary treatments such as bone marrow transplants won’t be necessary.
How we look after her now
After Kaydes recovered from her ear, we tried putting her back in daycare, but she got sick within two days.
So it was very apparent that she couldn’t go anymore. So between mum and myself, we look after her at home. It’s a bit of a balancing act between our jobs, and I’m so appreciative to both places for being so caring and accommodating.
Again what we thought might be a few months of being this way, might actually be quite a bit longer.
Day to day, she looks normal, just a bit on the pale side. She’s on 5ml of iron each day to help with the blood production, which I had hoped would’ve made an impact.
We take her outdoors all the time so she can get fresh air, and some new interaction play and sensory development. We just take extra precautions, such as:
- Sanitising hands, dummies, and bottles
- Avoiding crowds, or trying to go to the shops during slow times
- Sometimes going the long way around, to get away from crowding
- No playing with other kids (but watches some of them play occasionally 😭)
- Wearing sunscreen
- Wiping things down before she touches them
Immunisations are still recommended, so we’re getting her 1 year ones very shortly. And we’ve now got an appointment next Thursday with a visiting geneticist and her paeds specialist.
Still not out of the woods yet, but she’s been a remarkable and brave lil girl through it all. Even when she’s having an off day, she wants to happy, and loves bopping along to music. I just love Kaydes so much, and hope she’ll be ok ♥️
Lore & Tempo is where motherhood meets adventure. I’m Ren – storyteller, explorer, and single mum in Wellington, New Zealand. I’ve been lucky enough to wander through 45 countries. 15 of them with my +1, Kaydes, either strapped to my front, on my hip, or running ahead on her own adventures. I created this space to share single parent life, travel tips, and the joy of exploring the world.
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