Daff Family Session | Peas in a Pod By Karin Nagel

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Daff Family

I had the absolute pleasure of meeting, working with and photographing this beautiful family…

But let me start at the beginning and share their story with you (trigger warning..long and emotional post, it’s heartbreaking)

I came to learn about the Daff family and the harsh reality that they are facing, through a mutual friend Clare Clarke (she is also an amazing soul and one of the better PT’s out there!). Clare mentioned to me one day that she feels heartbroken and proceeded to tell me why:

This beautiful family, friends of hers, have received the most awful news. A diagnosis of Metachromatic Leukodystrophy in 2 of their beautiful children and my heart broke into a million pieces! I knew I had to do something, because creating positive memories is so important and even more important at a time like this. I had to photograph them, freeze time for them, give them these precious memories to cherish forever.

daff famliy, daff famliy, daff famliy, daff famliy,

I must admit I wasn’t overly familiar or had a great deal of knowledge on the disease, so I had to do a bit of homework.

What is Metachromatic Leukodystrophy?

Metachromatic leukodystrophy (MLD) is one of a group of genetic disorders called the leukodystrophies, which are characterised by the toxic buildup of lipids (fatty materials such as oils and waxes) and other storage materials in cells in the white matter of the central nervous system and peripheral nerves.

Symptoms include muscle wasting and weakness, developmental delays, progressive loss of vision leading to blindness, impaired swallowing , mental deterioration, an inability to control movements, seizures, and dementia. Symptoms continue to get worse, and death generally occurs within 6 to 14 years after onset of symptoms.

Is there any treatment?
There is no cure for MLD.

Bone marrow transplantation may delay progression of the disease in some infantile-onset cases. Other treatment is symptomatic and supportive. Considerable progress has been made with regard to gene therapy in an animal model of MLD and in clinical trials.

I couldn’t and cant even imagine what they are and will be going through and receiving such a diagnosis is a parents worst nightmare…

Can you imagine growing old with only 1 out of your 3 children?

Sam and Simons’ world has been turned upside down with Kenzie and Jackson facing this horrible genetic disorder, which sadly is terminal with no cure currently available.

No parent should ever have to watch a child go through something like this, let alone two. This disorder is extremely rare and the doctors who have diagnosed Kenzie & Jackson with MLD aren’t aware of any other cases in Western Australia.

I spoke to Sam on the phone and had to physically fight back the tears as I could tell in her crackly, over tired sounding voice, how heart broken she is. We didn’t waste time and got them scheduled for a family photo session on a very warm and sticky Sunday afternoon, but no matter what the weather would hold, we were going to do that session!

I am extremely lucky to have my wonderful husband by my side in helping me prepare for sessions like these, as I knew this was going to be emotionally difficult.

We met up with this beautiful family, mum Sam, dad Simon, Kenzie, 4, Ashlee, 2, and little Jackson, 6 weeks old…

The session itself was wonderful, it went down without any problems, even though all the girls wanted to do was see the kangaroos!

They were an absolute dream to photograph. Kenzie has a sense of divaness about her gorgeous self, as she filled the field with her beautiful little soul. And wonderful Ashlee showed a true sense of big sisterness, even though she is the youngest of the girls.

I asked Sam, “If there is one photograph she had to have, what would it be?” and she said: “The two girls together.”

I’m so pleased that I captured these beautiful sisters, being so happy in one another’s company and I hope this is an image that will be treasured forever.

daff famliy,
I quickly saw an opportunity to capture Sam and Simon together as a couple, and left my darling husband to babysit!

daff famliy,

Since finding out this horrible news, Sam and Simon have not had any time to just look at each other, hug and just re-affirm to each other that they are in this together. I saw this as an opportunity for them to talk, compliment and show appreciation to one another. Sure, there were a few tears, however I believe these words cannot go unspoken…love needs to be let out!

daff famliy,

So what do we know at this point in time?

Kenzie will be admitted to PMH hospital at the end of January/early February to undergo an unrelated umbilical cord transplant. This treatment has potential to alter the course of her disease and provide a better quality of life going forward.

The transplant process is unimaginably hard and commences with 9 days of intense conditioning (chemotherapy) followed by isolation for a number of weeks after her new cells have been transplanted.

daff famliy,daff famliy,

As for little Jackson, we are hoping that he will be eligible for Gene Therapy in Milan, Italy.

This therapy is still experimental but the results for pre-symptomatic kids is pretty exciting. They have been in touch with Milan and hope that they will be accepted on a new compassionate use program being set up as a result of the promising results from the 2010 trial. If accepted, this treatment will cost them $100,000US plus living expenses in Milan, Italy.

Side note: It’s important to note, that even though things have been revolving around Kenzie and Jackson, Ashlee has been such an amazing little person and continues to be such an amazing little person through all of this.

Even though Ashlee is only two, you get a sense that she knows something drastic is happening, and that her life will be altered, so in true Ashlee style, (bear in mind that she is the middle child) she is morphing into the most empathetic, compassionate, helpful and little protector to her siblings and a beautiful ray of sunshine!

daff famliy, daff famliy,

This is such an amazing family, and my husband and I feel so blessed and lucky to have met these wonderful people…

They have received an overwhelming amount of support from our local community, it’s wonderful to see that when times are hard, people can still come together. The Daff family has a gofundme page set up, so please follow this link for more information on updates, ways to help and donations.

With all the above in mind, I hope you take time to cherish the little things…the “insignificant” moments…as the harsh reality is, we just don’t know what tomorrow will bring.

 

 

2 thoughts on “Daff Family Session | Peas in a Pod By Karin Nagel

  1. Hi, having this diagnosis makes everything feel bitter sweet to the extreme, I know as my only child, my daughter, had a diagnosis of MLD last year. I researched and found also that in China you can get the same gene therapy as they do in Milan, but in the China trials they are accepting only children that have already started getting symptoms (juveniles with the slower progressing course), whereas in Milan they will not accept those children. Sorry for posting this message here, but I wanted to let the Daff family know and I am unable to post a comment on their news stories as I don’t have a facebook account as seems to be needed. In fact you don’t have to publish this comment at all, you could just pass my story onto them. All hope is a good thing. With love xxx
    https://clinicaltrials.gov/ct2/show/NCT02559830
    http://www.hku.hk/press/news_detail_12780.html

  2. Hi, having this diagnosis makes everything feel bitter sweet to the extreme, I know as my only child, my daughter, had a diagnosis of MLD last year. I researched and found also that in China you can get the same gene therapy as they do in Milan, but in the China trials they are accepting only children that have already started getting symptoms (juveniles with the slower progressing course), whereas in Milan they will not accept those children. All hope is a good thing. With love xxx

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