new diagnosis... unrealistic clinging to hope?

[OJL5-12-12]

Hi all...

So my 6 week old prince charming was diagnosed with CF last friday -11/01. There were a few pointers that indicated that he had a "health issue" as i now call it- the main one being his weight- he was born weighing 4lb15 6 days overdue. Just below the 0.4th centile. He didnt have his heel prick screen done on time - it was done 3 weeks late as the midwives had "overlooked it" =[ During this time there were 12 weighings were my little man appeared not to be putting much weight on- he was solely breast fed until 4 1/2 weeks. At this time he started putting weight on (8oz in 11days) was started on Creon on the 11th and has since put on another 8 oz in 6 days. So my reasoning is- there must be SOME part of his dogestive sustem working? He is homozygous df508 and i have only seen bad things assosciated with this?! It is so hard trying to get to grips with everything we need to know and all the meds etc...
I just feel like an inadequate mum at the minute because surely i should be able to make this right for my baby?! :'( any support or help would be greatly appreciated! Thanks so much x

 

[Aboveallislove]

Dear mom,

I'm so sorry for your pain. I lived that too, although with the newborn screen and finding out at 12 days old. Our son is ddf508. He was born at like 6% height and 10-20% weight (blur) and first CF appointment was around 9%?? At 3.5, he's now at 75% for height and 50% bmi. He has only had stomach issues so far. Not an ear infection, sinus infection, cold, flu, . . . anything. The "genotype" i.e. the genes he has, are only 50% of the phenotype, i.e., how his body handles the CF. And there are drugs that JUST last week got "breakthrough designation" from the FDA which could be ready for our littles ones in 2 years AND they have more in the works that 5 - 10 years out will be like a "cure." So your job now is to do the treatments and be proactive (and make sure no smoke, avoid sick folks, etc.) and so very soon there is something so much better waiting our littles ones. The hard part is now when you have all the new baby stuff AND the learning AND the fear, sadness, anguish. It does get easier and you'll be an old pro in no time!
Hugs and prayers,
Love

 

[Gorf]

Dear MOM,

Let me state that everyone of us is different. That said, I have both ddf508, diagnosed at 3 years old because of not gaining weight for a year and a half. My parents made me (mom helped me) do treatments until I left the home. I lived a normal life other than doing those treatments, and taking my meds for digestion. I had my first lung problem at age 19 with my first Pneumonia. With out giving you my whole life history, I am 47 years old now. Though my health has declined over the years, I am also waiting for the second part to this miracle drug. You as a parent will have hard days, but never give up hope. I lived a full life and now have a grandchild that is 3 years old with no CF, and my son did not have it either.

Gorf

 

[OJL5-12-12]

Thanks so much for the replies already!! Im just so stressed out- havent eaten slept or stopped crying for a week!! I just dont understand if he is as it says everywhere online pancreatic insufficient- how when we offered formula top ups he could have put weight on and his poops were becoming "tackier"?
Are these drugs a realistic hope for us all?! Is it the phase three kalydeco and vx809 combination that iv read of? Do we know the likely benefits of it?! So sorry for the million and one questions- just trying my best to know as much as possible about CF so i can help my gorgeous little boy. I know most parents with a child that has CF have probably all said it before me- but i need to have hope x

 

[Aboveallislove]

It is very very very unlikely he would be pancreatic sufficient with ddf508, but there might be a little functioning very earlier on. Our DS also gained when I added formula to the breastmilk. I would not think too much about the pancreatic sufficiency or not; you'll (and he) will get use to the enzymes in no time!

Yes, it is the Vertex combo 809/770. The drugs benefit all organs but this combo is just the first step. They have another in the works called 661 which is suppose to be better and they have discovered that a menu of correctors will be the future.

Hang in there. This time is truly the worst. You have all the "normal" post-partim going on to this normal reaction. I think I had a rosary imprint in my hand for 6 months b/c all I could do was squeeze and pray for so long. Also, some relaxing yoga helps--I like Gaiam (sp?) Am/PM yoga. . . it is mostly stretching and breathing and does amazing things to help. And please know this is normal. It will get better, but it is NORMAL.
hugs and prayers,
Love

 

[OJL5-12-12]

Oh its just so disheartening knowing the likelihood is that he is pancreatic insufficient because iv seen that those who have sufficiency tend to have less lung problems? I just hope to God that one day very soon (tomorrow would be ideal) we will be able to say- yep he has cystic fibrosis but its not going to affect his quality of life or indeed his life expectancy! I had always dreamed of such a fantastic stress free (as much as it can be) future for any children i was lucky enough to have (im sure we all do). It just kills me to think the chances are he wont outlive me (sorry for the morbid tone) to me- it feels as though my baby died when he was diagnosed and hes been replaced with this new one that needs so much more from us. It has taken all the joy out of nursing him (which i loved for the first four weeks)! So if im reading into these trials correctly- potentially they can reverse damage to the lungs and aid in the digestion of his food- is that accurate in lay man terms? Just so much to take on board- it annoys me so much i had the BF specialist out three times and i was throwing nappies in everyones faces- yet was told i was just an "overly anxious first time mum". =[

 

[Aboveallislove]

Lung function increases greatly on the drugs and a woman on Kalydeco (the drug approved for other mutations) said their CF doctors are now rethinking what "permanent" lung damage means because with the Kalydeco the lungs are regaining some function by clearing old mucus whereas the doctors had thought it was permanent damage. But these are folks much older--in there 30s. Yours has no damage yet. . . do the treatments and your best to keep it that way. The combo of drugs also shows improvement in vitro now to be on par with "milder CF." And there are other drugs (next generation) that will likely be ready for your little one by 6!!! Some on Kalydeco don't need enzymes any more after 20 years on them. . . others still do. The meds already here do much to keep the lungs stable and CFers healthy and the new meds will make their life long and healthy.

 

[OJL5-12-12]

We can but hope!! Iv read that the phase 2 results showed significant improvement in lung function with those who have at least one df508 and even greater improvement for those who are homozygous df508. I understand im still in that post natal period where things are "a bit off" anyway- and i can feel myself slowly becoming stronger with each day- i just find that if i see another child- i cry. If i see an elderly man- i cry. I know that he will be picking up on my emotional state right now and so im just trying my best to keep it together as much as i can. You all seem so strong and determined- i admire that so much!! I wish i was a stroger person!

 

[Ratatosk]

DS was born with a bowel obstruction caused by meconium illeus. Required surgery when he was born to correct that issue. We started doing chest physiotherapy (CPT) right away to keep his lungs happy and healthy, learned about giving him enzymes, nebulizer treatments.... Little by little we got into a routine, got on a schedule. Today, ds is a 9 1/2 year old -- goes to school, active in sports, went to daycare and preschool as a baby... Most of his issues are still digestive, some sinus problems and we continue to do cpt 3 times a day to keep his lungs clear.

It's my understanding that the pancrease still has some functionality, albeit, it's still insufficient.

 

[franzie2984]

Try to not be discouraged preemptively, you never know what is going to happen in the future! Just to add my two cents I am homozygous for df508 mutation and almost 30yrs of age with more pancreatic insufficiency issues than anything else (and I spent my entire childhood avoiding doing any lung treatments or medication other than enzymes). My lungs are pretty good and I am healthy. I also think most people with CF are somewhat pancreatic sufficient/partially insufficient, most people have some functionality. People vary in their symptoms and being homozygous for df508 doesnt necessarily mean anything in particular for your babys future. good luck!

 

[bigstar]

I'd say relax. You have a healthy child at the moment. You will have to redefine "normal" and 'healthy" from now on so be prepared. Enjoy the nursing, enjoy the first steps, the first words. Hope stands for everyone. Im 23 and im still dreaming a healthy life. Im still hoping that one day i wont be having to explain my situation, my cough , my taking meds and everything. Im still daydreaming of the day that im leaving for vacation without nebs, flutter and emotional burden. Hang in there. Dont give up on hope.

 

[Printer]

OJL5-12-12

Please don't cry when you see me. I will be 73 tomorrow (1-19) and last Sept we celebrated our 50th Wedding Anniversary.

Bill

 

[Aboveallislove]

Dear Mom,
Two other thoughts:
1) Wanted to make sure you are going to an approved CF Center and being seen by a CF doctor. The CFF cff.org has a list of approved centers--there are only about 110. It is very important to be seen at one.
2) If you want to post what your currently being prescribed, I'm sure folks would chime in on any ideas to look out for. Right now, at a minimum, your little one should be having a bronchial dialator (albuterol or Xopenex--I'd recommend Xopenex or the now available generic leverbuterol because it has less "shakes), percussions (2x pera day at mimimum), and special CF vitamins. And if there is any vomitting/acid reflux, Zantac.
Hugs and Prayer,
Love

 

[OJL5-12-12]

Hi everyone- thanks for all the info- hes currently on creon micro, vitamin supplements and flucloxacillin. We are having the physitherapist come out on monday to show us how to give Oscar his physio- she came this week but i was too much of a wreck for her to want to show me anything!

Printer- may i wish you a very happy birthday for tomorrow- i hope you get everything you wish for

Hes been very unsettled today- think he has a cold coming on- have heard him sneezing a fair bit these last 72 hrs! Even went so far as to ring the hospital as he has a bit of a temperature so obviously in my overly anxious mind the poor thing isnt even allowed to have a cold EVER!! Im trying my best to focus on the positives but there is just so many negative stories to be read online =( he has a CF team in the north west with a respiratory nurse, consultant, dietician and psychologist. We have arranged a few fundraisers already- we just feel like we need to be proactive otherwise we just sit and cry

 

[Murphysmama]

Dear Mom,

My heart breaks for you and your family. We were in your place a little over two years ago. I too focused on the fact that he was gaining some weight wo enzymes and the pi topic. Those early days were so so tough. You are a great mom!! You are already seeking advice and that's huge. I know everyone is different, but I would like to pass along what helped me initially in practical terms. First, there is so much out of your control and it's so complicated (the science and what it means and what it means for your precious baby). What really helped me was to focus on what I could do. For me that meant not reading a lot about the science end at first but focusing on the practical elements of care. I started a spreadsheet and recorded everything (wet/dirty diapers per day, enzyme dose and time, vitamins, physical therapy, antibiotics etc). It helped me keep track of everything I needed to do each day and I would get so many questions from doc that it gave me a feeling of being 'on top of it' at least in the daily care dept. The care that you provide for your child has a HUGE impact on their health. I was frustrated by the lack of good predictors for the course of CF for my son. The bottom line is there are so many factors that's impossible to nail down. My ds has ddf508 and reading that it is 'severe' etc is terrifying. We don't know how it will be and it's scary. What we do know is that the care you provide has a huge impact and I took comfort in knowing there was a lot I could do. We are two years out and it's not easy but it is much much more manageable than we initially imagined. It has made me appreciate everything with my children in a more profound way. It may sound odd, but I see more beauty and kindness in other people. Focus on spending time every day with your baby every day on just baby stuff. Holding head up, eye contact rolling over etc. you don't want to miss all of those precious moments bc CF has overwhelmed you. Watching ds hit the milestones has been very encouraging a d helps me focus on the fact that he's a little boy first.
Be gentle with yourself and let others help you. It's tough but before you know it you will find what works for you.
My doc prescribed hormone patches
Sending lots of hugs and light!

 

[Murphysmama]

Sorry the last part got cut off...
but the hormone patches can help even out the swings. They are tough enough on their own. I didn't end up using them but it was good to have a plan in place in case I did.

 

[Helenlight]

Hi,

Our baby is 14 months now, she's DDF508 also. I remember acutely those first months of overwhelming helplessness, they were some of the lowest moments of my life (and my partner's), and yet- we found a way through. You get past the frustration with the meds, you get past feeling like your dreams have been shattered, you get past pity and disbelief. Once you see your child getting bigger and growing strong, you will get a sense of hope and relief. You will be able to enjoy your natural parent/child relationship. Getting to that point is wonderful. You realise there is life after all and it is worth living! You begin to understand that while this diagnosis is a shock to the parents, to the kids it is simply the life they know, and we just gotta make the most of it. This is what my journey has been, and now I look at my magnificent daughter (who is now walking, almost running!) and see that she is enjoying life, and will continue to do so for a long time to come.

It's tough, and it's and eye-opener, but it's a window to a life with more gratitude and growth than you could ever imagine.

Look after yourself, fall back on your team whenever you need to, accept help, cry when you feel like it... and then look at your beautiful baby and smile Give him every reason you can to feel good about life. Treat meds and treatments in a matter-of-fact way, they're just like brushing your teeth.

In terms of all the technical questions, they will be answered in time, no need to rush. Our daughter is 'pancreatic insufficient' but her enzyme results show that she still has some activity- right at the bottom end of 'normal'. Keep in mind the pancreas is not the only part of the body to produce enzymes, but it does produce the enzymes for digesting fat and protein, so the function for processing sugars and carbohydrates is still there. In terms of gaining weight on the formula, I believe there could be two reasons for this. Our specialist told us that sometimes the pancreas can take some time to 'block up', (our girl was gaining normal amounts of weight on breast milk until she was 6 weeks old), and formula takes longer to digest and results in firmer stools- so some of that weight is likely to be food in the bowel- not necessarily body growth weight. Once you're on the right dosage of replacement enzymes and the correct amount of sodium chloride, it will be reassuring to see all that weight come on and your boy get healthy. I was able to breast feed for a full year before we needed any kind of prescribed supplementation (which may have helped prevent digestive issues).

Anyway, I wish you all the best! Focus on the positives where you can, keep trucking and you'll find yourself in a better place in no time.

xx

 

[Printer]

When I told you that I would be 73 today, I wasn't looking for your birthday wishes. I was telling you that CF is not a death sentence. At 73, i'm not even the oldest CF patient at my clinic. Your child will, most likely, have a full and long life ahead.

Bill

 

[OJL5-12-12]

Thats very encouraging to hear!! Its like the consultant told us- people who have CF and have long boring lives dont tend to put it online- most of the stuff you see online regarding CF is frightening and negative!! Im just overwhelmed at the moment by everything- but day by day i can feel myself coming back ready to fight for my boy

 

[Printer]

If you stay out here for a while, you will hear from alot of CF patients in their 50's, 60's and some in their 70's. A year ago, while a patient at the Massachusetts General Hospital, a nurse told me that they had a CF patient in the hospital who was in his 90's.

I know that it is hard and scary but try to relax. You have blown this situation way out of proportion. Talk to your CF Team at your Approved CF Clinic. It will be ok, believe me.

Bill