The Year Ahead


At the end of this last year in November we were fortunate enough to have an appointment with a biochemical disease doctor, Dr Galina Nesterova, who just so happens to have a lot of experience treating people with cystinosis. It was so amazing to finally be able to talk with an expert in the field and feel like my concerns were being properly addressed. Don’t get me wrong, I think that our nephrologist and all the support people we see at the renal clinic are great but sometimes I’ll bring up an issue which is fairly common among the cystinosis population and I’m met with resistance. I feel like my concerns get dismissed because they might think I don’t know what I’m talking about.

Here’s the thing though, I have far more time available to research cystinosis and the sorts of things I should be looking out for than they do. And I understand there’s a thin line between being cautious and overly paranoid when it comes to rare diseases but when I bring up a concern, I want to feel like I’m actually being listened to. For example I’ve been concerned about Elsie’s legs going a bit knock kneed and have been told several times from the renal clinic that it’s fairly common in children and they usually grow out of it by age 5, so until that age they don’t think action is necessary. However after bringing this up with Dr Nesterova she explained that for children with cystinosis the problem may not correct itself and could get worse as they age and gain weight. Then she referred Elsie for some physiotherapy and wants them to assess whether orthotics are needed to help keep her ankles from turning in. Finally, progress!

For the past few years Dr Nesterova has been assisting Dr William Gahl at the National Institutes of Health (NIH) in Maryland, USA in his Natural History Study of the Use of Cysteamine in the Treatment of Cystinosis and I’ve been wanting to take Elsie there since I first heard of it. Many people throughout America visit the NIH every couple years to take part in this study, which includes extensive testing and review from one of the top doctors in the field of cystinosis. But, living in Canada, I didn’t think it would be something we could participate in. However when I mentioned it to Dr Nesterova she said it would be no problem for us to participate and that we would just have to cover our travel costs! I was given the contact for the study coordinator and I’m hoping to bring Elsie there sometime in the Spring or Summer this year.

With the prospect of having experts in the field to ask questions from and get a real sense of just how healthy Elsie might be I’ve found myself looking to the year ahead with a lot of hope. Every year since Elsie’s diagnosis I’ve felt like we’ve gained more and more control of cystinosis. Instead of it running our lives it now feels like just a part of a our lives, and a manageable one at that. Sometimes I worry that this is just the eye of the storm and in a few years we’ll be thrown back into the chaos with something like her kidneys starting to fail, or maybe her thyroid will start having problems but for now I’m choosing to put all that fear aside and just live in this peaceful moment of health.

Her first check up at the renal clinic this year went fairly well but we have had to add some new medications to the mix. Her red blood cell count has started to drop so they’ve added a multivitamin called Renavite to try bring the count back up. They’ve also added an iron supplement called Euro-Fer and a half tab of Tums to add some calcium that she’s probably missing from her diet. At 15.53 kg she is sticking to her growth curve at the 26th percentile for weight and at 103 cm tall she’s in the 44th percentile for height which I think is awesome! All in all I feel like we’re in a good space at the start of 2017.

With the new additions to her medications I thought it’d be nice to post her medication schedule again:

1:30am – Cystagon 300mg

7:30am – Cystagon 300mg 

With Breakfast

  • Sodium Phosphate – 1.5ml
  • Potassium Citrate – 7.5ml
  • Spironolactone – 2ml
  • Carnitor – 2.5ml
  • Vitamin D Drop – 1

With Lunch

  • Potassium Citrate – 7.5 ml
  • Carintor – 2.5ml
  • Tums – ½ a tablet

1:30pm – Cystagon 300mg


  • Potassium Citrate – 7.5ml
  • Renavite – ½ a tablet
  • Euro-Fer – 2ml

With Dinner

  • Sodium Phosphate – 1.5ml
  • Potassium Citrate – 7.5ml
  • Spironolactone – 2ml
  • Carnitor – 2.5ml
  • Enalapril – 2ml
  • Vitamin D Drop – 1

7:30pm – Cystagon 300mg

At Bedtime – Growth Horomone

Eye drops – 6x/day: every two hours

Elsie has started to express an interest in learning how to swallow pills so hopefully soon we will finally get our act together and start teaching her how. It would if she could swallow pills when she starts Kindergarten in September. That way we won’t need to worry about medications needing to be refrigerated and things like that. When we do start the process I found this article from The Pursuit of Research with tips on how to teach your child to swallow pills which I think will be invaluable. And while I can’t believe my little girl will be starting school so soon, that I think is another post entirely!

All in all I’m looking forward to the rest of 2017. We hope to attend one of the cystinosis conferences this year as a family as I would love to meet and talk with some of the wonderful families we’ve met online and I think Elsie would love meeting and making friends with their beautiful children. So here’s hoping that 2017 meets up with my expectations.

New Year, New Medicine


New Year New Medicine

With the New Year ringing in I had high hopes that this year would be our best year with cystinosis. We’ve been living with it now for just over 2 years, have had lots of time to research, ask questions, learn about what everyone else is doing and coming to terms with it ourselves. So I was almost looking forward to our first visit of the year at the renal clinic (because who doesn’t love trying to contain a 3 year old in a tiny, sterile room for 2 hours) to see how Elsie’s levels were doing and how much more she’s grown.

The good news is that she continues to grow, at least in height. But unfortunately we just can’t get this girl to put on weight. Our doctor would like to see Elsie start to gain much more this year and I would love that too. Obviously her health is the number one priority but secretly I would also love to be able to buy her pants that fit her waist AND leg length. Right now she’s about a 4T in leg length but only a 2T in waist size. I know many people are probably shaking their heads at the shallowness of this but I’m telling you the struggle is real!

Anyway operation fatten Elsie up is officially on and so far I’m finding it difficult to brainstorm healthy ways to get more calories into her. She is so strong willed, I prefer to bang my head against a wall than to attempt talking her into trying something new. So in other words, you can forget about it! Sometimes she will tenderly touch the new food to her tongue, say it tastes good, but refuse to actually put it in her mouth. And our dietician is recommending that Elsie get about 1600 calories a day!

Basically our only option is to trick her into extra calories or work with what she does eat. Right now she loves eating butter, just straight up butter. Perhaps it’s time to just plop a stick of butter in front of her and be done with it? But I can’t imagine there are any long term benefits, or that she would feel very well after that. There is however a product called Duocal which is supposed to be a tasteless powder that you can add to food or beverages for added calories. We haven’t tried it yet but if it is indeed tasteless and doesn’t change the texture too much it would be perfect for adding to her water. We’ll be looking into where we can get it in the next couple days so I will update you guys on how it goes. And if anyone has any other ideas on how to trick a preschooler into more calories I’d love to hear them!

I’m also sad to report that another medication has been added to Elsie’s daily regime. The protein/creatinine ratio in her urine has increased significantly in the past 3 months, from around 300 mg/mmol to over 700 mg/mmol. For reference sake a normal range for someone without kidney disease should be less than 30! The amount of albumin in her urine is also quite high which we’ve been told suggests that not only are the tubules of her kidneys experiencing damage but now also the glomerulus.

To try and combat this additional damage to her kidneys our doctor has prescribed Enalapril, an angiotensin-converting enzyme (ACE) inhibitor that lowers blood pressure. Though she doesn’t have high blood pressure the hope is that Enalapril will lower the pressure in her kidneys so that they won’t have to work as hard and lessen the damage to them. Turns out that this is quite a common treatment to prolong the kidneys in chronic kidney disease patients so we feel good about adding it in with the rest of them. We’ll be testing her levels again in a few days just to make sure all is well so here’s hoping this will lower her urine protein/creatinine ratio.

2016 may not have started out as stellar as I was hoping, but it’s not awful either. The Enalapril will hopefully do it’s thing and getting some meat on Elsie’s bones is a good goal to have throughout this year. Luckily her brother is eating like a champ and then some so now she’s got someone to compete against and my girl loves to win!

Up, Up and Away

Up up and away

It’s been 2 months since we started Elsie on growth hormones and so far we are super happy about it! She’s currently 78.9cm (2.58 feet) tall and weighs 8.9kg (19.62 pounds)and is now just below the 3rd percentile for both height and weight. It’s so exciting to see her pant legs start to get a little too short and retire those pair of shoes she’s been wearing for almost a year. Her waist is still quite small so it’s hard to keep pants up on her but we’re still trying to fatten her up. Our doctor had mentioned that we might see an increase in her appetite after starting the growth hormones but I would say it’s only slightly increased so far. However at this point any increase is good!

The one down side to her growing is that now all of her medications have increased. While it’s sad to give her the higher doses, at least it means she’s finally catching up with the all the other toddlers. We’ve also had to add two more meds to her daily schedule, an antibiotic and a laxative. In Elsie’s 23 months of life she has already had 4 UTI’s andwhile our doctor told us this is not usually typical with cystinosis there have been quite a few parents online that stated this was common for their children when they were young. So to avoid further infections Elsie has been put on a low daily dose of antibiotic. Daveand I aren’t very keen on this and it seems that for others the UTI’s seemed to cease after potty training so we may try to do this sooner than we’d planned. On the plus side Elsie does have a potty and likes to use it occasionally! The laxative is fairly self explanatory, it’s to help keep her regular because she seems to go through waves of going everyday and then getting blocked up for a week or two. Our doctor also thinks there may be a correlation between her being constipated and getting so many UTI’s. I can tell you since starting the laxative potty training has become more attractive to us!

Below is what her medication schedule now looks like:

2am – Cystagon 125mg

8am – Cystagon 125mg

With breakfast
o Potassium Citrate 2.5ml
o Sodium Phosphate 1ml
o Lactulose (laxative) 5ml
o Cysteamine Eye Drops 1 drop per eye

2pm – Cystagon 150mg

With lunch
o Potassium Citrate 2.5ml
o Cysteamine Eye Drops 1 drop per eye

6:30 pm – Omeprazole (proton pump inhibitor) 3ml

With dinner
o Potassium Citrate 2.5ml
o Sodium Phosphate 1ml

At bedtime
o Somatropin (growth hormone) 0.7mg
o Novo-Trimel (antibiotic) 1.5ml
o Cysteamine Eye Drops 1 drop per eye

8pm – Cystagon 150mg

She is still taking all of her medications really well and actually tolerates the nightly injection of growth hormones better than her eye drops. I posted a video on Facebook of Elsie getting her injection because I thought it was amazing how quickly she recovered from it and was so proud of her. Reactions to the video were mixed though. Most people who were not familiar with cystinosis or seeing a small child get a shot found the video to be a little upsetting but those of us that have been exposed to it, for one unfortunate reason or another, thought it was really great how quickly she rebounded. Everyday I witness the amazing strength and courage that Elsie has and am constantly reminded that I should never underestimate her. Her happy go lucky attitude has brought so much love and positivity into our lives and I can’t imagine a world without her.

Dealing With Insurance


Dealing with Insurance Coverage

When it came to getting our insurance company to cover all of Elsie’s medications it was pretty much a nightmare. And it seems to be that way for a lot of cystinosis families, due to the rareness of the disease and it’s medications. After a couple months our insurance did finally cover all of her medications including her Pediasure, which is practically unheard of so I wanted to share our experience in the hopes that it will help others in a similar situation.

First of all talking to the people at their call centre was a complete waste of time. When I first called to see if her meds were covered all they would say was they were not covered under our plan. When I asked what about her medically necessary, life sustaining medications were not coverable they repeated they just weren’t covered under the plan. That was it. The only advice they had on how to proceed was to write a letter requesting them to re-assess our coverage.

So after speaking with our doctor and social worker at the renal clinic they offered to write a letter to our insurance company. We got two letters, one from our nephrologist stating why Cystagon and the cysteamine eye drops were medically necessary, and one from our dietician stating why Pediasure was critical to her overall health. The one from our dietician was pretty harsh even stating that without proper nutrition Elsie could die but they said that in their experience strong language was needed to get their attention and really drive home how important the Pediasure is. I put together a package with these letters, a cover letter stating what we knew would happen to Elsie without her medications and some facts about cystinosis and it’s treatment pulled from the Cystinosis Research Foundation’s website and sent it off to our insurance company.

A couple weeks later we got a letter back saying that although they were not questioning the validity of her disease and medications they could not be covered under our plan. I was furious! How could they say they understood how serious and important her medications were but still not cover them? I was sure that if Elsie needed certain medications to survive then they must be obligated to cover them but sadly I found out this is not the case. Insurance companies are in the business of making money and if they choose not to insure specific medications it is completely up to their discretion. How frightening is that?!

So I scoured through the benefit package that Dave received looking for anything that would indicate why her drugs would not be covered under our plan. All I could find were statements as to why they should be covered, such as the medications were necessary for the treatment of her disease and there were no generic alternatives. So again I called the call centre to argue my point and was told the same frustrating answer, they’re just not covered under our plan. But at least I was told that I should speak with Dave’s HR department for further clarification.

I sent off an email to the HR representative explaining our situation, what had transpired so far, and attached everything that had been sent to the insurance company and their reply letter. I stated that we were extremely anxious to get coverage for our little girl and would be willing to provide anything needed and even pay a higher premium if there was a more expensive plan that would cover her drugs. Finally it was in the hands of someone that actually gave a damn. First the HR rep enquired with BC Fair PharmaCare to see if her medications went towards our deductible.

In BC there is a program called Fair PharmaCare which all households need to apply for, you’re not just automatically enrolled. What they do is give you a deductible based on your family income that resets at the beginning of each year. Once you reach this deductible they will cover 70% of your medications until you reach your maximum (also determined by your income) and then they will cover 100%. This is to ensure that the cost of your families medications won’t bankrupt you. As an example our deductible is $1400 and our max is $1878, it’s now mid June and we have already reached our max level for the year. So for the rest of the year all of our medications will be covered 100% by Fair PharmaCare. Here’s the kicker though, not all medications are approved under this plan. In order to have Elsie’s medications covered our doctors had to apply to Health Canada to request a special authorization to bring her medications into the country and have them covered our provincial health plan. Due to the rareness of cystinosis, Cystagon is not available in Canada nor FDA approved. And her cysteamine eye drops are made specially for her by the hospital pharmacy, apparently they need to be made in the same sterile environment that they make IV’s.

Back to obtaining coverage, we informed the HR rep that Elsie’s medications were in fact covered under Fair PharmaCare because special authorization was applied to and granted by Health Canada. We asked our social worker if she could send us the paperwork which proved this was the case and she gladly forwarded it along. We then sent it to the HR rep who forwarded it on to their claims manager at the insurance company and awaited a response with bated breaths. We were worried because we only had special authorization paperwork for Cystagon as the eye drops were made just for Elsie at the pharmacy. We had already assumed that the Pediasure would not be covered as I did find it stated in Dave’s benefit package that infant formula would not be covered but due to the large volume Elsie needs to consume this is one of our highest expenses so I still wanted to push for it.

Lo and behold about a week later we received an email for the claims manager that Cystagon, the cysteamine eye drops and Pediasure were all going to be covered! It was incredible the sense of relief and lightness I felt with that email. After all our battles and frustrations the outcome was better than we had hoped. The whole process took about 3 months and coverage has only been approved until November 2014 as that’s how long Heath Canada approved her meds for but I’m very optimistic that they will be covered again.

After that getting her Omeprazole and growth hormones covered was easy, we just needed to supply the special authorization approval letter to our claims manager and they were both approved. I should mention that in between Elsie starting her meds and approval from the insurance company Variety Children’s Charity gave us a grant to fully cover her meds until we reached our Fair PharmaCare deductible. Without their support we would’ve had to rely on the financial assistance of our parents but we hated to ask them for it. Variety does amazing work for families and children in need so if you have a moment to donate to this worthy cause it would be greatly appreciated. One could argue that we didn’t need to fight with our insurance company if we already had this grant but I was determined to make our insurance pay for her meds as that’s the whole reason for insurance! And I wanted to free up the grant money to other families, like us, in need who may not have been as lucky as us in ensuring coverage.

Step Up

Step Up

This Mother’s Day we wanted to do something really special for Elsie’s grandmas because they have been two of the most amazingly supportive, understanding and loving women not only throughout Dave and I’s lives but also through this period of transition as we adjust to life with cystinosis. Although a gift can never really honour and thank our mother’s the way they deserve I think we came pretty close, and as you can see in the picture above Elsie approves!

Step Up For a Cure are these adorable, personalized, wooden stools made by a family in Ontario. A perfect stepping stool for little ones, they’re sturdy and can be decorated anyway you like. We had the phrase “Grandmas hold our tiny hands for just a little while but our hearts forever” and a picture of the backs of two teddy bears hugging painted on our stools. The price per stool ranges from $40-$50 and the best part is that all of the proceeds go to the Cystinosis Research Foundation! The family started this project in 2012 shortly after their beautiful daughter was diagnosed with cystinosis, so if you’re in need of a stepping stool (or a time out stool) in your house or the homes of your relatives I hope you will consider these one of a kind stools. Elsie’s grandmas loved theirs!

Step Up For a Cure

For an Elsie update she has been doing really well lately! We just received the results of her white blood cell (WBC) cystine test and it’s 0.82! The WBC cystine test is the test that officially confirms if someone has cystinosis. A person without cystinosis will have a level right around 0 but when Elsie was diagnosed her WBC cystine was 2.26. After starting cystagon at 400mg per day her WBC was tested again a couple months later and it came back at 1.13, which was a definite improvement but the aim is to get the level below 1, although between 2 and 1 is still beneficial. So her cystagon was upped to 500mg per day and tested again 3 months later, which came back at 0.82. We’re super happy that her cystagon doesn’t need to be increased any further and that her cystine level is once again safe.

We’re also getting close to starting growth hormones. We’ve been registered with the Saizen program (Momentum in Canada) and received their Easypod in the mail which is a sort of high tech injection system. The pharmacy has already filled her prescription for growth hormones but we are just waiting to hear about coverage from our insurance company before going to pick them up. This is because the cost is over $300 just for two weeks! And if all goes well for Elsie on growth hormones she could potentially be using them for years. Once we get the coverage figured out, hopefully in the next week or so, a nurse will be coming to our house to show us how to use the Easypod and we’ll be good to go. Fingers crossed she doesn’t mind the nightly injections.

We’ve also just enrolled in a local infant development program. We were referred to an occupational therapist for feeding therapy to get some tips on how to increase the amount Elsie eats but the wait list is quite long. So they offered to put her in this development program as we wait. Initially I didn’t want to do it because we don’t have any developmental concerns with Elsie and I didn’t want to put her under even more of a microscope. But while at the CRF Day of Hope Conference Dr Angela Ballantyne did a talk on Quality of Life Issues in Nephropathic Cystinosis which looked at the the potential academic and motor problems that can occur with cystinosis. In it she talked about the little things that we can do to encourage development in our children which could help make their futures easier. During this talk I realized that my resistance to the infant development program was more because I didn’t want to have another thing to worry about as opposed to wanting to protect Elsie from more tests. So we will be visited by a counselor in a couple weeks to discuss our goals and learn how we can encourage Elsie to continue learning and developing at a healthy pace.

As always Elsie continues to be her happy, smiling self which is all we can ask for! Now go buy a stool 😛

The Importance of a Second Opinion

Importance of a Second Opinion

After the whole NG tube business I had written an email to our renal clinic nurse saying that we were sick of not being listened to and in the future we needed to be better informed about why these decisions were being made. After some back and forth between us we had sorted everything out. From their point of view they were listening to us but honestly felt that the NG tube was the best way for Elsie to get her nutrients and the amount of water she needed. From my point of view I was frustrated that we went ahead with the NG tube when we were sure that it was not going to work and I was upset that I didn’t have the guts to say no. One of the things that came from this exchange was that they recommended we get a second opinion from another Nephrologist. Immediately I felt bad because we never felt like we were getting sub-standard care from our Nephrologist and besides the NG tube we were very happy with her. But I knew the doctor they were recommending has been seeing another girl with cystinosis for 12 years now so we were looking forward to meeting with him and seeing what new knowledge and care we could get from him.

We are SO happy that we met with him. Right away he was just a wealth of information specifically about cystinosis and because of that he was able to look at Elsie as an individual with cystinosis. Before then we felt like Elsie was being treated as a typical cystinotic but we weren’t so sure that she was. There are so many stories out there about these children having an unquenchable thirst and soaking through diapers in the matter of an hour and Elsie has never been like that. We’ve been trying so hard to get 1.5 litres of liquid into her when it turns out that drinking enough liquid is not usually a concern for cystinotics because they just can’t get enough of it. So finally after months of trying to explain that Elsie just didn’t seem that thirsty someone finally got that! He was amazed at how she went a full 20 minutes without going to her water bottle and wants to do some more tests to see if Elsie has maybe a mild form of cystinosis or perhaps a new CTNS mutation that hasn’t been seen yet.

He was also shocked that she wasn’t wearing sunglasses in such a bright room. Photophobia is a very common problem for cystinotics because of the cystine crystals that form on their corneas and while Elsie seems to have a bit of a sensitivity to light it doesn’t seem to be as extreme as in some cases of cystinosis. This symptom I think varies pretty widely among cystinotics though. But he mentioned that in his career he’s probably seen about 10 to 15 different cases of cystinosis and almost all of those patients needed to at least wear sunglasses when outside and we’ve never been able to get Elsie to keep sunglasses on for more than a few seconds. Thankfully though she seems to be coming around to them now that she’s a little older because cystinosis or no cystinosis I still worry about her eyes!

And then he asked us if we’d heard of Procysbi, the delayed release form of Cysteamine, which only needs to be taken every 12 hours as opposed to Cystagon which must be taken every 6 hours. Obviously we have heard of it and would love to get Elsie on Procysbi but it is not yet approved in Canada and the cost is astronomical at over $250,000/year! However one of his patients is in a Procysbi trial out of Stanford University and as part of the trial the medication is completely covered. We actually met with this wonderful young woman and her mother in January when they were in town and although Procysbi is already approved in the US, they will continue to supply her with it until it becomes available in Canada which is just amazing for them. So he offered to contact the study on our behalf to see if they were taking new patients. I’ve since heard back from him and unfortunately they are not but he says that there may be other ways to get Procysbi to us and he will continue to keep his eyes and ears out for us if any other trial becomes available. Although I have already registered Elsie with the Cure Cystinosis International Registry and on the Cystinosis Research Network to be notified if any trials come up that she would qualify for it is amazing to know that there is someone in the medical profession looking out for Elsie too.

Sweet Girl

Finally we talked about how Elsie is doing body wise. Her labs came back looking really good with all of her electrolytes in their desired range and due to this fact he thinks that she is getting an adequate amount of nutrition and liquids which was so awesome to hear. However she didn’t grow too much more since her last visit. She’s still in the 0.1 percentile for both weight and height but she’s not growing as well as they had hoped. So he is recommending we try growth hormones. Growth hormones have become a fairly standard practice for children with cystinosis to try and help them catch up with the growth curves. Many adults with cystinosis tend to be much shorter than their parents. I had actually brought up growth hormones with our first Nephrologist and she had told me it wasn’t really something they would look at yet since Elsie is so young so I was surprised to hear this new Nephrologist recommend them. But if it will help Elsie get back on that growth curve Dave and I are all for it. However administering growth hormones requires a nightly shot which may not go over too well with Elsie, but we think that it’s at least worth a try. So there are some more tests Elsie will need to do to make sure growth hormones are safe and could work for her, which we will probably get done before we see them next in April. The really exciting thing is that at this time he is recommending the growth hormones over the G tube. Which makes Dave and I so happy because we’d like to put off the G tube surgery for as long as possible.

We are so grateful we were given the opportunity to get a second opinion and see someone who has had some real experience with cystinosis. Even though we didn’t feel like our first Nephrologist was that bad now that we’ve seen someone with experience we can see that the difference is huge! If I have any advice for other families out there with cystinosis it would be to try and seek out a medical professional who has treated patients with this disease before. I know that it can be quite difficult since it is such a rare thing but for our family it was been amazing, and that has just our first visit. Once again I am looking forward to Elsie’s future with some more positivity, hooray!

And the NG Tube is Out

Elsie and Lambie

Our family saga with the NG tube is at an end. It was only in for a week but it felt like years and I have to say it was the most trying thing we’ve been through during this whole cystinosis thing.

The insertion of the tube went pretty much as we thought. They swaddled her up in a blanket and one nurse tried to hold her head still and another inserted the tube. She screamed, cried, choked and gagged and it was pretty awful to witness. The worst part was taping it to her skin because she was so upset at that point and all I wanted was to hold her. And it didn’t help that she was due for a nap. After insertion we cuddled as I tried to calm her down. Over the next hour we learned how to feed her through the tube and we quickly realized that we were in way over our heads.

There is so much work involved in NG tube feeding that no one bothered to tell us about before hand. Before putting anything through the tube you need to make sure that it’s still in the stomach. So first you have to push a bit of air down the tube to make sure it’s clear, then you pull the syringe up to remove some stomach contents. You then need to test it on a pH strip to make sure it’s acidic enough, which means the tube is still in the stomach. Then you can insert water or formula through the tube. If it’s formula, after a feeding you need to flush the tube with around 15ml of water to clean it, and then you need to push some more air into the tube to clear the water out. Oh and all of this Elsie could feel as it went through the tube and into her stomach and she did not like it!

She spent the first hour in fits of tantrums. She’d be calm for a few minutes and then she’d try to yank the tube out, so I’d hold her hands down which would make her scream and cry for a minute and then she’d be calm again… for a few minutes. Eventually we just had to kick everyone out of the room so we could try get her to nap. Once all the lights were out and everyone left she fell asleep in my arms pretty quickly but would wake up and cry everytime I tried to put her down so I ended up holding her through most of her nap, which was only about 40 minutes. After she woke up they re-taped the tube to her face, which was awful, but it was much more secure and she no longer had anything to grab at. After that she was much better and by the end of the day Dave and I thought that this might actually work.

But once we got home and tried putting some formula down her tube it was all over. She absolutely hated having anything to do with the tube. She cried every time she saw her reflection, or anytime attention was brought to it. After speaking with our nephrologist on the phone she recommended that we hold off putting anything through the tube while she adjusted to just having it in. The problem was she never really adjusted. She was constantly rubbing her face where the tape was and she just wasn’t the same little girl anymore. She was much quicker to throw a tantrum, had lower energy, was more clingy to me in particular and wasn’t nearly as happy although she did still have her moments which were nice.

Although we weren’t putting anything through the tube we still had to flush it with water to keep it clean. You’re supposed to do it every 6 hours but even that was too much for Elsie to handle. We were lucky if we could do it twice in a day. The problem was she would get so upset that she’d start gagging and I was terrified of her throwing the tube up because then we’d have to put it back in again.

There is a plus to all of this though. Turns out she likes drinking from giant syringes. When we first had the syringes ready to go to feed through the tube she grabbed one, brought it her mouth and started drinking. Usually once she finished a 60ml tube of formula off she would ask for more! It was amazing and I had no idea she could fit that much liquid into her stomach in one go.

So after a week with the tube we were still barely using it, Elsie was still miserable and Dave and I were both at the end of our ropes. I even had to call my mom in to help because I was so stressed out and depressed about the whole thing I could barely handle looking after Elsie. I sent an email to our renal clinic nurse asking if we could meet with our nephrologist and discuss the possibility of taking out the tube and the reply I got was keep trying with the tube and our doctor was not available. For some reason this email just broke me. I haven’t felt that depressed and hopeless about the whole situation since she was diagnosed. I decided that it was time I really took a stand for Elsie’s care and started doing what I felt in my heart was right.

So we took the NG tube out. It wasn’t a decision that we took lightly. We looked at all the pros and cons and in the end agreed that there were far more cons than pros to keeping the tube in. The big deciding factor for us was that through Elsie’s new found love of drinking liquid from syringes we were now getting very close to the recommended 1.5 litres of fluid into her per day. And it was way easier feeding her orally than through the tube. The difference once we took the tube out was immediate. We took it out just before dinner and by the end of the night we had our happy, lively little girl back. I can honestly say that I have no regrets from our decision to take out the tube.

We meet with the surgeon tomorrow to talk about getting a G tube inserted and after the whole NG tube business I am much more on the fence about this procedure. But thanks to our experience with the NG tube Dave and I now know what questions to ask and what we need to know in order to make a better, more informed decision regarding Elsie’s care. The path right now is still very stressful and bumpy and my outlook as of late has not been very positive but I know that although there are hard decisions to be made as long as Dave and I keep Elsie’s best interests in mind and have the support of our family everything will be okay.

The NG Tube is Out