Lipoprotein Lipase Deficiency (LPLD) Community

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6 year old diagnosed with LPLD

Hi everyone,

I am the dad of football loving 6 year old who had a 2nd episode of pancreatitis and the root cause on the deck is LPLD. The docs ordered genetic testing to confirm the source.

The triglycerides went from 550 to 1100 the next day during the hospital stay after eating a little amount of Pasta....

We are still coming to grips with this. My son seems to have fully recovered from the event.

Any advice on what to expect/what to eat and how to adapt will be great?

Also what questions/tests/treatment options should we be asking the doctors? Do you also recommend any experts in the Mass/New England area?

Assuming he follows a low fat diet, what are the chances of the kid having a normal childhood?

--Vish

sandyketronvowell
moderator

He absolutely CAN have a thriving childhood and life.
I have been in low fat diet my entire life. With good choices to protect the pancreas, it's possible that he could have no further episodes.

My suggestion is to locate an endocrinologist.
Go to our FCS website and get a copy of the booklet to take w you to doctor. Read as much as you can to educate yourself.

If you are on Facebook, we have a pretty strong presence there.

Living with FCS

So sorry for what your family is going through. But there IS help and hope.

Sandy in TN

jill
moderator

Hi Vish, good to see you here. I can imagine that the diagnosis has been a huge shock and surprise and I'm glad that you've found us.

Sandy is right, your son can have a thriving childhood and it's a good idea to join the facebook community that she mentions. The best support can often comes from people who really understand what it's like to live with this condition.

Making the right choices for your son and teaching him the importance of making the right choices as he grows older and more independent is key to living well with LPLD.

Please look at the UK based charity, LPLD Alliance www.lpldalliance.org which has lots of tips about living with LPLD (http://www.lpldalliance.org/support/self-m...

They may help you to begin to think about how to approach what lies ahead.

We also have a facebook presence and a closed group - LPLD Discussion community -which has a number of parents with children (some of them football-loving boys!) who will be very supportive, including a couple of dads. If you message from the LPLD Alliance facebook page I can add you. Many people are members are both groups as many of the issues we deal with go beyond borders!

If you have any questions about the condition, this page or about any of the information above please don't hesitate to ask, and if you accept my friendship request you can privately email me if you'd prefer.

Jill

Thank you so much Jill and Sandy.

My son is back in the hospital and he had a second episode that started yesterday. He had a good 4 day period before landing in the hospital again. The Trig's are 1830 and the Lipase was >500 last night. Today it dropped to 300.

He seems to be ok. They are doing an MRCP now and also they are talking about feeding food using a tube into the Intestines directly. I have not heard about that and I am sort of scared at this point. Is this something we can do at home?

They also found Pseudo cysts and I will find more info from the labs later today. Thanks for the support. I finally found a place to find some peace during this storm of uncertainty.

By the way I accidentally clicked the "Report" button, please ignore it. It has been a crazy week....

--Vish

jill
moderator

Hi Vish, I'm really sorry to hear your son is back in hospital. I'm hope he's not too uncomfortable. I can imagine you might be feeling a little overwhelmed.

I am not a medical professional so I can't give advice but my immediate reaction to the idea of a feeding tube straight into the intestine is that it's the content of the food that's the problem in LPLD, ie fat, not the means by which it is delivered, so I would be very wary about the need of having a feeding tube straight into the intestine - a gastrostomy. If it's really necessary, because of a different problem, I would ask if it's possible to have the far less invasive nasogastric tube in the first instance.

I have experience of my son having brain cancer and I knew many children with nasal gastric tubes (which he had) as well as gastrostomies, and it is certainly something that can be managed at home. However I would really try to get some sense of what the rationale is for going straight for this surgical procedure without trying less invasive options first, or whether such an option is necessary at all.

Are you on facebook? If so I recommend you joingnthe two facebook pages as you may find others who have had a similar experience and the conversation is much more active than here on rare connect.

sandyketronvowell
moderator

Oh my. I completely had an "oh no" when I read the feeding tube. As Jill said, our problem is the fat content in the food. TRADITIONAL method for treating pancreatitis is no food at all, put on IV saline solution until triglycerides go down in order for your pancreas to rest.

Please consider another discussion with your medical team.

Have you gone to the FCS Foundation site to fine the booklet for FCS people to learn the basics about the disease?
Go to this website: https://fcsfocus.com/diet-lifestyle/
You can DOWNLOAD the book immediately, and you can ask for one to be mailed to you.

My trigs run 2500 at the lowest. Even following my diet.

The main thing. The MAIN thing --- is to manage the symptom of pancreatitis.

Are you seeing an endocrinologist?
A lipid specialist?
A pancreas specialist?

Any of these should be able to access the tools necessary to treat your son.

If they are not open to listen to your thoughts on the problem, then they are not part of your team.

I strongly suggest you get with others of us who have this disease. Join our discussions. Please join us on Facebook.

Sandy Ketron Vowell

jill
moderator

Hi Vish, how are things today?

Great couple of days. The Lipase levels dropped to 76 and TG's dropped to 600 with no intervention. On a clear liquid diet now. The MRCP shows a small 2.5 / 2.4 fluid cyst on the Head of Pancreas potentially causing a narrowing of the duct. Overall the kid is passing all pain tests so far. The fluid cysts are small enough to be absorbed on their own as pancreas recovers.....

Day 1 - 1890 TG's and Lipase >500(unknown)
Day 4 - 600 TG's and Lipase 76 now....

Hoping to keep the TG's in the range of 600 to 700 to not trigger another episode.
I also read about Volanesorsen drug in trials. Will have to see....

jill
moderator

Hi Vish, it's great that your son is doing so much better and had no intervention. Phew!

Please consider joining the two facebook discussion groups (if you use facebook). You'll be very welcome.

FCS Support Group based in the US and LPLD Discussion Community here in the UK.

These are the places where you'll be able to access support about how to manage the food that your son eats so that he doesn't have another attack.

If you don't use facebook, feel free to ask questions here on the forum.

Best wishes to you and your son.

Jill