Categories
Uncategorized

‘I don’t like the Cystic Fibrosis, why must I have it?’ – mom talks about her 4-year-old’s Cystic Fibrosis journey

Rynah was born in October 2016. She was a normal, healthy baby and I had an easy birth – there were no complications.

Her growth and weight gain were normal up until the age of about 9 months when she got bronchiolitis and a cold not long after that, but it wasn’t anything too serious. I did notice her breathing was a bit laboured, so I took her to the doctor who put her on antibiotics.

After this she started getting ear infections all the time, and her chest starting acting up a lot, but both always cleared up after a course of antibiotics.

We have asthma in our family so we just assumed her chest issues was related to this.

ALSO SEE: Asthma, what you need to know

She was doing well for a while but then she had issues with her chest again and was coughing. She had just started at a new little school so we put it down to her being exposed to more germs. But her cough just kept on getting worse and worse.

In March 2017, when Rynah was about 16-months-old, I decided to make an appointment with her original paediatrician in Milnerton where she was born.

The paediatrician took an x-ray of her chest and it looked as if something was stuck in her chest. There was this white spot on her right upper lobe and they weren’t sure what it was.

Little Rynah and Jolene

We were eventually referred to Dr Fiona Kritzinger, a paediatrician and paediatric pulmonologist who did a sweat test on Rynah to test for Cystic Fibrosis (CF). I didn’t think much of this test at the time because at that hospital they do the sweat test on every little patient that comes in. But I knew about a child with CF in the Blouberg area, so I was aware of how serious it is.

The first sweat test came back inconclusive. A second sweat test also came back inconclusive and we then had to do a genetic test. The test came back positive for delta F508 – which is the most common mutation of Cystic Fibrosis and technically the most severe.

We then also had to test our son (who was 5 or 6-years-old at the time) to make sure that he doesn’t have it.

Rynah’s CF journey was very weird in a way. She recovered well; her physio went well – she was very strong.

But a month later she got a fever and two days later she got very sick and had to be hospitalised again. She had RSV this time, but she recovered quickly and was fine again within 2 days. We then had a whole good year of check-ups all the time, but she still had this persistent cough often at night.

ALSO SEE: RSV is on the rise – here’s how to tell the difference between this virus and COVID-19

When we went for her check-up in March the following year, the x-ray showed that her right upper lobe had collapsed entirely. She needed a lobectomy.

Rynah had the operation to remove the lobe last year October just before her third birthday and my husband and I were terrified. But even though she looked terrible with all the pipes and tubes coming out of her little body, the operation went well and there were no complications.

Rynah after her operation

Within 4 days after the op she was running around again, kicking balloons as if nothing had happened. It’s actually quite insane how strong she is.

Since she’s had the op, she hasn’t been hospitalised once, she hasn’t coughed once again or even been really sick once. She has also not cultured staph (an infection caused by bacteria commonly found on the skin or in the nose) again and her X-rays look almost normal.

She just did her first ever lung function test (which is quite difficult for a 4-year-old) and she scored 98. For a person missing a lobe this is amazing as a 100 is normal.

She is in school for the first time – proper school with 5 kids.

Rynah healthy

So, we’ve had a good year. But the reality is that CF is not something that stops. We’ve seen how progressive it can be. We don’t know what her X-ray will look like by next year. We don’t know if it was just the staph that caused all the damage or if it’s just how progressive her CF is. Every person’s Cystic Fibrosis is very different.

ALSO SEE: ‘You fight today, just for another day for your child to breathe’ – mom opens up about battle to get life-saving meds for 3-year-old

This year we struggled a lot with her tummy. She is severely pancreatic insufficient, which means her pancreas doesn’t produce any enzymes at all. We still don’t know how she gained weight – we suspect it’s because I breastfed because it’s not normal to grow.

Life-changing medicine

I follow many of the medications that are released for CF patients by Vertex. Orkambi was the first one released. When Rhynah was born, Orkambi was just released and Americans were already using this medication. Rynah qualifies 100% for this medication because it is for the specific strain she has. So, she could already be on modulators which stops the progression of CF. It doesn’t cure it, but it means no further damage and no lung transplant one day.

She also qualifies for the next level medication called Symdeco. Patients who’ve used it have seen amazing results. They have now started using it on 6-year-olds and are trailing it on younger kids as well. She could have been on that medication by next year already and then had a chance of living a normal life.

A lot of women become infertile because their cervix is clogged up with mucous. On Trikafta, within 2 months women can fall pregnant because that has changed. Some people who have used Trikafta have gone from being on lung transplant lists to not being on one – this is all the people who’ve already had a lot of damage. What they are finding with the younger ones is that those who are diabetic stop needing insulin because all of a sudden, their pancreas is working. A lot of things just start working normally.

The stats show that the younger you start on these medications, the more you can prevent the progressiveness of this disease. Because as you get older, when hormones start kicking in, that’s when the progression starts speeding up.

A lot of kids are healthy up until around 12 or 13 years. There’s a boy on this one CF group I belong to – he is 10-years-old and he is on his last. They can’t do anything for him anymore. They are just keeping him comfortable now.

This year we lost so many kids between the ages of 12 – 18 years who died because they don’t have access to meds like this.

At the moment, when other people look at Rynah, she looks healthy – she looks like a walking miracle, but the reality is that it’s an internal thing. It keeps on going.

So, with Trikafta once again, she qualifies 100% for that and it would mean that she can live a normal life expectancy. It would mean she could become a mom. She wants to be a mom. She’s only 4 but she understands that already.

Rynah with her doll

She asks me things like ‘Mom, why do I have to nebulize every day?’, ‘Why do I have to do it twice a day?’, ‘Why do I have to cough?’ – she’s only 4 and she’s already questioning it.

She says to me: ‘I don’t like the Cystic Fibrosis, why must I have it? Why must I have tummy pains?’

Getting this medication would mean less treatments for her and a normal, fulfilling life.

We don’t want the medications for free, we just want access to it, but we also need to be able to pay for it. It has to be fair.

Leave a Reply

Your email address will not be published. Required fields are marked *