Recently Joe had several tests and chest x-rays for his annual review at hospital, we will receive the results for these at the beginning of June. During this visit Joseph’s medication was also changed and increased quite substantially. We are just getting used to the changes so thought it would be useful for me to write it all out with his new schedule but also might be useful for raising awareness of how much medication people, even babies, who suffer with Cystic Fibrosis have to have on a daily basis just to function normally.
In a previous post I set out an explanation of some of his medication- you can see that here.
Joseph now no longer takes Dalivit (which is a multivitamin for children) instead he takes:
This medicine is a multivitamin supplement and is used in the prevention of vitamin deficiencies in people who have conditions such as galactosaemia (a genetic disorder that causes intolerances and difficulty feeding amongst other things) , phenylketonuria (Phenylketonuria (PKU) is a rare genetic condition that is present from birth. The body is unable to break down a substance called phenylalanine, which builds up in the blood and brain.) or other metabolic disorders.
Joseph has to take this medication because he cannot get vitamins from food due to his pancreas not functioning correctly. He also struggles to absorb vitamins from this medication so is given additional medication to try and supplement this. He has a much higher dose of vitamins than other people because of his inability to absorb them (the dose Joseph takes would be very dangerous to other babies of his age and he is monitored closely). Vitamin deficiencies cause huge amounts of problems- too many to list here- so this medication is very important and vitamin medication will play a large role for the rest of his life.
This medicine contains vitamin A, ergocalciferol (vitamin D), cyanocobalamin (vitamin B12) and choline chloride. Vitamins are complex chemicals that are required in extremely small quantities for a variety of body functions. The body cannot make these vitamins itself and has to obtain them from food.
Alpha Tocopheryl acetate
This is a man made form of vitamin E.
Vitamin E plays a vital role in maintaining cell structure and is known to have strong antioxidant properties.
Natural vitamin E is fat-soluble and is normally absorbed into the body with fats consumed in the diet. A deficiency in vitamin E may occur if there is a problem with absorption of fat (malabsorption disorders), such as in cystic fibrosis.
The body normally produces bile to help it absorb fats from the gut, so in conditions where there is a problem with the production of bile, for example in certain liver diseases, or if there is a blockage of the bile duct, there can also be problems absorbing fats and fat-soluble vitamins.
Vitamin E suspension is given to people with these sorts of disorders to provide the body with extra vitamin E and thus correct deficiencies of this vitamin.
- Chronic liver disease, causing neurological deficits
- Gross lack of coordination of muscle movements with loss of deep tendon reflexes
- Truncal and limb ataxia
- Loss of vibration and position senses
- Paralysis of extra-ocular muscles responsible for eye movements
- Muscle weakness
- Ptosis (drooping upper eyelid)
- Dysarthria (motor speech disorder)
- Slow growth in children
Ketovite tablets contain a combination of ascorbic acid (vitamin C), thiamine (vitamin B1), riboflavin (vitamin B2), nicotinamide (vitamin B3), calcium pantothenate (vitamin B5), pyridoxine (vitamin B6), biotin (vitamin B7), inositol (vitamin B8), acetomenaphthone (a form of vitamin K), alpha-tocopheryl acetate (vitamin E) and folic acid.
These tablets again help Joseph absorb much needed vitamins into his body as he cannot get them from food.
Menadiol is a vitamin K supplement for people who cannot absorb sufficient vitamin K from food. Vitamin K is a fat-soluble vitamin which is essential for blood clotting. A lack of vitamin K can lead to unwanted bleeding. Vitamin K helps with bone health also. Joseph is at risk of diseases such as osteoporosis (a weak bone disorder) amongst other conditions due to his inability to absorb vitamins hence why this medication is very important.
Sodium Chloride Solution
This is basically liquid salt. Because of Josephs Cystic Fibrosis he loses a lot of salt from his sweat especially in hot weather. He is encouraged to eat food with a higher salt content than other babies and during the months of May-September he is give Sodium chloride to keep his salt levels up. Salt helps maintain the fluid in our blood cells and is used to transmit information in our nerves and muscles. It is also used in the uptake of certain nutrients from our small intestines. The body cannot make salt and so we are reliant on food to ensure that we get the required intake. If Joe does not get enough salt he is at high risk of Hyponatremia- symptoms include nausea and vomiting, headache, confusion, lethargy, fatigue, loss of appetite, restlessness and irritability, muscle weakness, spasms or cramps, seizures, and decreased consciousness or coma.
The following is Joseph’s average daily medication and treatment schedule (when he is poorly this is increased/changed depending on what’s wrong)
Inhaler/ nebuliser (3 puffs, ten seconds between each)
Physiotherapy (around twenty minutes including percussion and active playing)
Mezzopram/ Omeprazole (1 tablet crushed on apple sauce)
Sodium Chloride solution (0.8ml)
Menadiol (1 tablet crushed on apple sauce)
Ketovite solution (5ml)
Ketovite tablet (one crushed into food)
Alpha Tocopheryl Acetate (1ml)
Creon Micro (around 2 scoops on apple sauce to digest cereal, then another scoop on apple sauce for yoghurt)
Milk with gaviscon and extra creon micro (2 doses)
Physiotherapy (around 10 minutes including percussion and active play)
Domperidone (1.4 ml) ideally 30 mins before food
Ketovite tablet crushed with lunch
Creon micro (several doses depending on fat content in food usually 1 scoop per snack, 2-3 scoops with meals)
Milk with gaviscon and extra creon (2 doses usually)
(high calorie snack needed between meals where possible- and extra creon)
Physiotherapy (see above)
Domperidone (1.4ml) ideally 30 mins before food
Ketovite tablet crushed with tea
Creon micro (see above)
(High calorie snack needed and extra creon)
Physiotherapy (see above)
Sodium chloride (0.8ml)
Inhaler (3 puffs)
creon micro (see above)
milk with gaviscon and extra creon
Looks like a hell of a lot when you write it down! And believe me we are not perfect, its pretty tough trying to fit all this stuff into one day around meals and nappy changes and snacks and everyday life so we do sometimes unintentionally miss things but we are working on it!
Hope you find this useful/ interesting!
xxxx love Rach, Rob and Joseph